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Sample records for hemidiaphragmatic agenesis presenting

  1. Presentations of agenesis of the hemidiaphragm in an adult

    International Nuclear Information System (INIS)

    Izeldin, Osama M.; Ahmed, Mohamed E.

    2008-01-01

    A 67-year-old man was presented with chest tightness and vomiting of one-week duration. On physical examination bowel sounds were heard on the left chest. Plain chest x-ray, barium swallow and CT gastric scan suggested volvulus with diaphragmatic hernia. At laparotomy there was complete agenesis of the left hemidiaphragm with no diaphragmatic remnants seen and no associated lung hypoplasia. This is an extremely rare condition and careful assessment is needed to differentiate between diaphragmatic hernia and agenesis of diaphragm as more cases could be diagnosed in the future as a result of good perinatal care of agenesis of hemidiaphragm (AHD) and long term survival to adulthood. (author)

  2. Agenesis of the dorsal mesentery presenting in an adolescent

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    Anith Chacko

    2013-03-01

    Full Text Available Agenesis of the dorsal mesentery is a rare occurrence that usually presents in children. It is associated with proximal small bowel malrotation as well as high jejunal atresia with discontinuity of the small bowel. We present a case report of an adolescent presenting with clinical features of proximal small bowel obstruction (confirmed on imaging as well as acute pancreatitis. At laparotomy, he was found to have no dorsal mesentery, without small bowel atresia, and the duodenum was fixed to the posterior abdominal wall. The patient recovered well and remained symptom-free.

  3. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

    International Nuclear Information System (INIS)

    Griffin, N.; Mansfield, L.; Redmond, K.C.; Dusmet, M.; Goldstraw, P.; Mittal, T.K.; Padley, S.

    2007-01-01

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution

  4. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Mansfield, L. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Redmond, K.C. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Dusmet, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Goldstraw, P. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Mittal, T.K. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-03-15

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution.

  5. A Case of Distal Vaginal Agenesis Presenting with Recurrent Urinary Tract Infection and Pyuria in a Prepubertal Girl.

    Science.gov (United States)

    Dural, Ozlem; Ugurlucan, Funda Gungor; Yasa, Cenk; Bastu, Ercan; Eren, Hulya; Yuksel, Bahar; Celik, Serdal; Akhan, Suleyman Engin

    2017-02-01

    Isolated distal vaginal agenesis is a rare anomaly and mostly becomes symptomatic after menarche. We describe an unusual presentation of this anomaly in a prepubertal girl. An 11-year-old prepubertal girl presented with recurrent urinary tract infection, pyuria, and right-sided renal agenesis. The findings of perineal inspection, ultrasonography, and magnetic resonance imaging were consistent with a distal vaginal agenesis with pyometrocolpos. Discharging pyometrocolpos with dissection of the atretic portion and a pull-through vaginoplasty were performed. A cystoscopy showed no sign of a vesicovaginal or uterine fistula. This rare presentation of distal vaginal agenesis reminds us that congenital malformations of the female genital tract should be considered in patients with congenital anomalies of the urinary system and/or recurrent urinary tract infection, even during the prepubertal period. Copyright © 2016 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  6. Lumbosacral agenesis in a cat

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    Gabrielle C Hybki

    2016-01-01

    Full Text Available Case summary Lumbosacral agenesis is a rare congenital condition reported in children. We report a 17-week-old female domestic shorthair cat with lumbosacral agenesis on whole-body radiographs. The cat was euthanized shortly thereafter presentation. A necropsy was not permitted. Relevance and novel information This is the first reported feline case of lumbosacral agenesis.

  7. Neurological presentations, imaging, and associated anomalies in 50 patients with sacral agenesis.

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    Emami-Naeini, Parisa; Rahbar, Ziba; Nejat, Farideh; Kajbafzadeh, Abdolmohammad; El Khashab, Mostafa

    2010-10-01

    Sacral agenesis is an uncommon congenital disorder that involves multiple organs. We studied neurological manifestations of the disease, common associated disorders, and their relation with extent of bony malformation. We investigated neurological manifestations of 50 patients with sacral agenesis. Patients were evaluated for previous procedures, ambulation, limb abnormalities, vertebral alignment, recurrent urinary tract infection, urinary incontinence, dribbling, dimple, lower extremities weakness, myelomeningocele (MMC), and lipomyelomenangocele. Weakness of lower extremities was seen in 37 (74%) patients. Concurrent weakness of proximal and distal muscles of the lower limb was statistically associated with a type of bony aplasia (P = .001). However, paraplegia was seen in only 2 of 44 children over the age of 1, and the rest could walk. Myelodysplastic syndromes were seen in 21 patients. Sacral agenesis is diagnosed in children with concomitant MMC at younger ages and reveals more severe symptoms. Progression of neurological disorders was seen in 19 patients, in all of whom MRI showed tethering of the spinal cord. Urinary disorders including diurnal urinary incontinence (in 30 of 35 children over age 4) and recurrent urinary tract infections (in 37) were also common. Imperforate anus was seen in 11 patients. Twelve children over age 4 reported fecal incontinence, a problem that had statistically significant association with imperforate anus (P = .013). Different disorders can concurrently affect patients with sacral agenesis that may have profound impressions on patients and their families. Early diagnosis, thorough evaluation, and proper intervention are of utmost importance as they can prevent or lessen future complications.

  8. A rare case of Turner′s syndrome presenting with Mullerian agenesis

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    Suresh Vaddadi

    2013-01-01

    Full Text Available Turner′s syndrome also called as Ullrich Turner′s syndrome, is a disease of unclear pathogenesis characterized by complete or partial absence of one sex chromosome, with or without cell line mosaicism in a phenotypic female with short stature. Various anomalies result in a constellation of features, of which the most disturbing is primary amenorrhea due to gonadal dysgenesis. Hormone therapy in these patients can often result in successful menstruation, and scope for subsequent pregnancy because of anatomically normal uterus and vagina. Coexisting Mullerian agenesis in these patients can jeopardize the chances of future pregnancy as they have associated structural abnormalities of the uterus and vagina. We report a rare case of middle-aged female with Turner′s syndrome and Mullerian agenesis having absent secondary sexual characters and missing uterus with incompletely formed vagina.

  9. Pulmonary agenesis

    OpenAIRE

    Oyola, Mercedes; Pontificia Universidad Javeriana; Gordillo, Gisel; Pontificia Universidad Javeriana; García, Carlos A.; Pontificia Universidad Javeriana; Torres, David; Pontificia Universidad Javeriana

    2009-01-01

    Pulmonary agenesis is an infrequent pathology which occurs predominantly among females with no lateral preference. We report on the case of a newborn male diagnosed with prenatal diaphragm hernia though at birth seemed more likely either to be a congenital cystic adenomatoid malformation (congenital pulmonary airway malformation) or pulmonary agenesis. The patient died six days after birth and necropsy confirmed pulmonary agenesis. La agenesia pulmonar es una alteración poco frecuente, con...

  10. Congenital agenesis of unilateral parotid gland with ipsilateral type I first branchial cleft anomaly: A rare presentation

    OpenAIRE

    Tripti Maithani; Apoorva Pandey; Seema Acharya

    2014-01-01

    Aim: To report a rare case of unilateral parotid agenesis with ipsilateral type I first branchial cleft anomaly. Material and methods: A case study with special emphasis on the embryology, outlining the complex developmental process of parotid and branchial arches and highlighting the probable reason for development of such anomalies. Results: The literature states that unilateral parotid agenesis is a rare entity with few reported cases occurring solely or in conjunction with other hea...

  11. Accessory hepatic lobe simulating a left hemidiaphragmatic tumor

    International Nuclear Information System (INIS)

    Kuroiwa, Toshiro; Hirata, Hitoshi; Iwashita, Akinori; Yasumori, Kotaro; Mogami, Hiroshi; Teraoka, Hiroaki

    1984-01-01

    A 72-year-old woman with a 20-year history of neuralgia was confirmed at surgery to have a tumor in the left hemidiaphragmatic region which was connected with the left lobe of the liver. Reassessment of radiological diagnosis after surgery revealed that hepatobiliary scintigraphy and computed tomography using left anterior oblique scanning are useful in differentiating the accessory hepatic lobe of the liver from a tumor and in confirming the diagnosis, respectively. (Namekawa, K.)

  12. Biallelic PMS2 Mutation and Heterozygous DICER1 Mutation Presenting as Constitutional Mismatch Repair Deficiency With Corpus Callosum Agenesis: Case Report and Review of Literature.

    Science.gov (United States)

    Cheyuo, Cletus; Radwan, Walid; Ahn, Janice; Gyure, Kymberly; Qaiser, Rabia; Tomboc, Patrick

    2017-10-01

    Constitutional mismatch repair deficiency syndrome is a cancer predisposition syndrome caused by autosomal recessive biallelic (homozygous) germline mutations in the mismatch repair genes (MLH1, MSH2, MSH6, and PMS2). The clinical spectrum includes neoplastic and non-neoplastic manifestations. We present the case of a 7-year-old boy who presented with T-lymphoblastic lymphoma and glioblastoma, together with non-neoplastic manifestations including corpus callosum agenesis, arachnoid cyst, developmental venous anomaly, and hydrocephalus. Gene mutation analysis revealed pathogenic biallelic mutations of PMS2 and heterozygous DICER1 variant predicted to be pathogenic. This report is the first to allude to a possible interaction of the mismatch repair system with DICER1 to cause corpus callosum agenesis.

  13. Pulmonary agenesis: two cases reported

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    Denis Yaraví Solano-Vázquez

    2014-11-01

    Full Text Available Background: Pulmonary agenesis is a rare anomaly (1 in 15 000 live births which consists in a total absence or severe hypoplasia of one or both lungs. The clinical spectrum of the unilateral agenesis could vary from early and severe respiratory distress, recurrent pneumonia to being an incidental finding. The prognosis is based on the presence of associated congenital abnormalities. Material and methods: We present two cases of unilateral pulmonary agenesis in patients at Tlaxcala’s Children Hospital during 2012. Results: Report details the case of a one-month old boy with left pulmonary agenesis and interatrial communication and mild pulmonary arterial hypertension. He had two resolved pneumonia incidents. The other case was a one-month old girl with right pulmonary agenesis, associated to multiple heart malformations who evolved to respiratory failure, heart failure and death.Conclusions: Pulmonary agenesis is a rare anomaly. Its outcome and prognosis varies with the hemodynamics related to its location and associated malformations.

  14. Klippel-Feil syndrome with associated agenesis of lung and gall bladder presenting with asthma and allergic rhinitis

    International Nuclear Information System (INIS)

    Khanna, Puneet; Panjabi, Chandramani; Shah, Ashok

    2005-01-01

    Klippel-Feil syndrome (KFS), a triad of short neck, limitation of neck movement and low posterior hairline, is characterized by the presence of congenitally fused cervical vertebrae and is often associated with multiple congenital anomalies. A 35-year-old male was referred for evaluation of an 'opaque hemithorax'. This led to a diagnosis of KFS, agenesis of left lung and gall bladder. The patient had history of wheezing dyspnea with nasal symptoms, which were diagnosed as asthma and allergic rhinitis. A high index of suspicion is required to recognize such a patient, and efforts should be made to seek other congenital anomalies. (author)

  15. Maxillary sinus agenesis - report of two cases

    International Nuclear Information System (INIS)

    Pierre, Jorge Henrique Arraes de Alencar; Santana, Expedito Araujo

    2000-01-01

    Agenesis or aplasia of the maxillary sinuses is an extremely rare condition, and only eight cases have been reported in the world medical literature. These malformations may arise as a result of developmental defects. Two cases of unilateral agenesis of the maxillary sinus are presented and the radiological abnormalities and the embryology are discussed. The literature is also reviewed. (author)

  16. Gallbladder Agenesis with Refractory Choledocholithiasis.

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    Tjaden, Jamie; Patel, Kevin; Aadam, Aziz

    2015-01-01

    Congenital agenesis of the gallbladder is a rare anomaly which is usually asymptomatic and found incidentally. In some cases, however, patients are symptomatic. Common symptoms include right upper quadrant abdominal pain, nausea, and vomiting. Jaundice is present in some symptomatic cases and is due to associated choledocholithiasis (Fiaschetti et al. 2009). In this case, a 63-year-old female presents with jaundice and episodic right upper quadrant abdominal pain with nausea and vomiting. Bilirubin and alkaline phosphatase were found to be markedly elevated. Upper endoscopic ultrasound (EUS) revealed choledocholithiasis, and the patient required multiple endoscopic retrograde cholangiopancreatography (ERCP) sessions before successful extraction of all stones. Subsequent surgical exploration revealed congenital agenesis of the gallbladder. Although this is a rare finding, patients with agenesis of the gallbladder are at increased risk of developing de novo choledocholithiasis which may be challenging to extract.

  17. Gallbladder Agenesis with Refractory Choledocholithiasis

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    Jamie Tjaden

    2015-01-01

    Full Text Available Congenital agenesis of the gallbladder is a rare anomaly which is usually asymptomatic and found incidentally. In some cases, however, patients are symptomatic. Common symptoms include right upper quadrant abdominal pain, nausea, and vomiting. Jaundice is present in some symptomatic cases and is due to associated choledocholithiasis (Fiaschetti et al. 2009. In this case, a 63-year-old female presents with jaundice and episodic right upper quadrant abdominal pain with nausea and vomiting. Bilirubin and alkaline phosphatase were found to be markedly elevated. Upper endoscopic ultrasound (EUS revealed choledocholithiasis, and the patient required multiple endoscopic retrograde cholangiopancreatography (ERCP sessions before successful extraction of all stones. Subsequent surgical exploration revealed congenital agenesis of the gallbladder. Although this is a rare finding, patients with agenesis of the gallbladder are at increased risk of developing de novo choledocholithiasis which may be challenging to extract.

  18. CT of tracheal agenesis

    International Nuclear Information System (INIS)

    Strouse, Peter J.; Hernandez, Ramiro J.; Newman, Beverley

    2006-01-01

    Tracheal agenesis is a rare and usually lethal anomaly. In the past, opaque contrast medium was injected via the esophagus to demonstrate the anatomy. To demonstrate the utility of helical and multidetector CT in delineating the aberrant anatomy in newborns with tracheal agenesis. Four newborns with tracheal agenesis were identified from three institutions. Imaging studies and medical records were reviewed. Each child was imaged with chest radiography. One child was imaged on a single-detector helical CT scanner and the other three on multidetector scanners. Helical and multidetector CT with 2D and 3D reconstructions clearly delineated the aberrant tracheobronchial and esophageal anatomy in each infant. Minimum intensity projection reformatted CT images were particularly helpful. One infant each had type I and type II tracheal agenesis. Two infants had type III tracheal agenesis. All four infants died. CT is a useful tool for delineating the aberrant anatomy of newborns with tracheal agenesis and thus helps in making rational clinical decisions. (orig.)

  19. Agenesis of pancreas

    DEFF Research Database (Denmark)

    Voldsgaard, P; Kryger-Baggesen, N; Lisse, I

    1994-01-01

    Complete agenesis of pancreas is a rare and lethal condition. Four cases have previously been reported in combination with other malformations, such as severe intrauterine growth retardation, hyperglycaemia and meconium ileus. We report a case of pancreatic agenesis as a single anomaly. The child...

  20. Gallbladder Agenesis with Refractory Choledocholithiasis

    OpenAIRE

    Tjaden, Jamie; Patel, Kevin; Aadam, Aziz

    2015-01-01

    Congenital agenesis of the gallbladder is a rare anomaly which is usually asymptomatic and found incidentally. In some cases, however, patients are symptomatic. Common symptoms include right upper quadrant abdominal pain, nausea, and vomiting. Jaundice is present in some symptomatic cases and is due to associated choledocholithiasis (Fiaschetti et al. 2009). In this case, a 63-year-old female presents with jaundice and episodic right upper quadrant abdominal pain with nausea and vomiting. Bil...

  1. Isolated bilateral congenital lacrimal gland agenesis – Report of two cases

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    Manar Alwohaib

    2017-10-01

    Full Text Available Congenital lacrimal gland agenesis, also called congenital alacrima, is a rare cause of dry eye and is characterized by aplasia or hypoplasia of lacrimal glands. We present two 5-year old children with congenital lacrimal gland agenesis. The two cases had the final diagnosis of isolated bilateral congenital lacrimal gland agenesis and we document the clinical aspects, treatment and present a literature review related to this rare condition. Keywords: Alacrima, Lacrimal gland, Lacrimal gland agenesis, Punctal plugs

  2. Early hemi-diaphragmatic plication through a video assisted mini-thoracotomy in postcardiotomy phrenic nerve paresis

    Science.gov (United States)

    Tsakiridis, Kosmas; Visouli, Aikaterini N.; Machairiotis, Nikolaos; Christofis, Christos; Stylianaki, Aikaterini; Katsikogiannis, Nikolaos; Mpakas, Andreas; Courcoutsakis, Nicolaos; Zarogoulidis, Konstantinos

    2012-01-01

    New symptom onset of respiratory distress without other cause, and new hemi-diaphragmatic elevation on chest radiography postcardiotomy, are usually adequate for the diagnosis of phrenic nerve paresis. The symptom severity varies (asymptomatic state to severe respiratory failure) depending on the degree of the lesion (paresis vs. paralysis), the laterality (unilateral or bilateral), the age, and the co-morbidity (respiratory, cardiac disease, morbid obesity, etc). Surgical treatment (hemi-diaphragmatic plication) is indicated only in the presence of symptoms. The established surgical treatment is plication of the affected hemidiaphragm which is generally considered safe and effective. Several techniques and approaches are employed for diaphragmatic plication (thoracotomy, video-assisted thoracoscopic surgery, video-assisted mini-thoracotomy, laparoscopic surgery). The timing of surgery depends on the severity and the progression of symptoms. In infants and young children with postcardiotomy phrenic nerve paresis the clinical status is usually severe (failure to wean from mechanical ventilation), and early plication is indicated. Adults with postcardiotomy phrenic nerve paresis usually suffer from chronic dyspnoea, and, in the absence of respiratory distress, conservative treatment is recommended for 6 months -2 years, since improvement is often observed. Nevertheless, earlier surgical treatment may be indicated in non-resolving respiratory failure. We present early (25th day postcardiotomy) right hemi-diaphragm plication, through a video assisted mini-thoracotomy in a high risk patient with postcardiotomy phrenic nerve paresis and respiratory distress. Early surgery with minimal surgical trauma, short operative time, minimal blood loss and postoperative pain, led to fast rehabilitation and avoidance of prolonged hospitalization complications. The relevant literature is discussed. PMID:23304442

  3. Isolated Unilateral Pulmonary Artery Agenesis complicated by Symptomatic Aspergilloma

    LENUS (Irish Health Repository)

    Daly, A

    2017-11-01

    Isolated unilateral pulmonary artery agenesis is a rare diagnosis. Poor blood flow to the lung parenchyma renders the tissue susceptible to opportunistic infections. We present the unusual case of isolated unilateral pulmonary artery agenesis complicated by aspergilloma. Management options and considerations are discussed.

  4. Two cases of sacral agenesis

    Energy Technology Data Exchange (ETDEWEB)

    Choi, J Y; Bae, Y K; Hahm, C K; Kang, S R [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    1979-06-15

    Sacral agenesis is a central component in the spectrum of anomalies comprising the caudal regression syndrome. Sacral agenesis occurs more frequently in an infant born to mother of diabetes, rubella infection in first trimester than normal mothers. In a patient of sacral agenesis, it is important to recognize the neurologic deficit, neurogenic bladder dysfunction and other congenital anomalies. A case of partial sacral agenesis of 10 years old girl and another case of complete total agenesis associated with multiple anomalies in autopsied newborn are reported.

  5. Two cases of sacral agenesis

    International Nuclear Information System (INIS)

    Choi, J. Y.; Bae, Y. K.; Hahm, C. K.; Kang, S. R.

    1979-01-01

    Sacral agenesis is a central component in the spectrum of anomalies comprising the caudal regression syndrome. Sacral agenesis occurs more frequently in an infant born to mother of diabetes, rubella infection in first trimester than normal mothers. In a patient of sacral agenesis, it is important to recognize the neurologic deficit, neurogenic bladder dysfunction and other congenital anomalies. A case of partial sacral agenesis of 10 years old girl and another case of complete total agenesis associated with multiple anomalies in autopsied newborn are reported.

  6. Gallbladder agenesis in a Chihuahua.

    Science.gov (United States)

    Kamishina, Hiroaki; Katayama, Masaaki; Okamura, Yasuhiko; Sasaki, Jun; Chiba, Satoshi; Goryo, Masanobu; Sato, Reeko; Yasuda, Jun

    2010-07-01

    A 4-year-old neutered male Chihuahua was presented with a history of anorexia and vomiting. Continuous elevation of liver enzymes was found on repeated blood examinations and the dog was referred to us for further evaluation. The absence of gallbladder was suspected on ultrasonography. Exploratory laparotomy and retrograde cholangiography confirmed gallbladder agenesis and a possible hypoplasia of the right medial and lateral liver lobes. Histologically, proliferation of bile ductules associated with portal fibrosis and pseudolobular formation were apparent in the liver lobes.

  7. Complete dorsal pancreatic agenesis and unilateral renal agenesis.

    Science.gov (United States)

    Moreira, Adriana; Carvalho, André; Portugal, Inês; Jesus, José Miguel

    2018-02-01

    Dorsal pancreatic agenesis is a very rare congenital anomaly. Unilateral renal agenesis, on the other hand, is a relatively common congenital anomaly, although its etiology is not fully understood. Renal and pancreatic embryologic development appears to be nonrelated. We report a case of a 34-year-old man who was referred to our hospital for evaluation of cholestasis and microalbuminuria. Ultrasound and magnetic resonance imaging examinations showed empty right renal fossa and absence of the pancreatic neck, body, and tail. Our case report is the second case of a dorsal pancreatic agenesis and unilateral renal agenesis in a young male patient.

  8. Sirenomelia with agenesis of corpus callosum.

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    Shirani, Shapour; Rekabi, Vahab; Kamalian, Naser

    2006-07-01

    Sirenomelia is a very rare anomaly presented with fusion of the lower limbs. Genitourinary, neural tube, and vertebral anomalies are found in most cases. We report a case of sirenomelia with agenesis of corpus callosum, which has not been reported previously.

  9. Postmortem MRI of bladder agenesis

    Energy Technology Data Exchange (ETDEWEB)

    Barber, Brendan R. [St George' s Hospital, Radiology Department, London (United Kingdom); Weber, Martin A. [Great Ormond Street Hospital for Children, Department of Histopathology, London (United Kingdom); Bockenhauer, Detlef [Great Ormond Street Hospital for Children, Department of Nephrology, London (United Kingdom); Hiorns, Melanie P.; McHugh, Kieran [Great Ormond Street Hospital for Children, Radiology Department, London (United Kingdom)

    2011-01-15

    We report a 35-week preterm neonate with bladder agenesis and bilateral dysplastic kidneys. A suprapubic catheter was inadvertently inserted into one of the larger inferior cysts of the left dysplastic kidney. A postmortem MRI scan was performed with the findings being confirmed on autopsy. We are unaware of another postmortem MRI study demonstrating bladder agenesis. (orig.)

  10. Postmortem MRI of bladder agenesis

    International Nuclear Information System (INIS)

    Barber, Brendan R.; Weber, Martin A.; Bockenhauer, Detlef; Hiorns, Melanie P.; McHugh, Kieran

    2011-01-01

    We report a 35-week preterm neonate with bladder agenesis and bilateral dysplastic kidneys. A suprapubic catheter was inadvertently inserted into one of the larger inferior cysts of the left dysplastic kidney. A postmortem MRI scan was performed with the findings being confirmed on autopsy. We are unaware of another postmortem MRI study demonstrating bladder agenesis. (orig.)

  11. [A case of lingual agenesis].

    Science.gov (United States)

    Carinci, F; Felisatti, P; Curioni, C

    1996-01-01

    Lingual agenesis is a rare anomaly caused by failed morphogenesis of the lateral lingual swellings during embriogenesis. Most reported cases have been part of oromandibular limb hypogenesis syndrome (OLHS). A case of lingual agenesis associated with micrognathia and alteration of the hands is reported.

  12. Agenesis of the corpus callosum with associated inter-hemispheric ...

    African Journals Online (AJOL)

    Adele

    developing countries should be alert to organic disorders presenting with ... CT Scan (Figures 1 and 2) of the head revealed agenesis ... co-existing lipoma is often seen with this type of ... childhood behavioural disorders, bipolar disorders,.

  13. Isolated Left Pulmonary Artery Agenesis: A Case Report

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    Tansel Ansal Balcı

    2012-08-01

    Full Text Available Unilateral pulmonary artery agenesis without any cardiovascular malformation is a rare anomaly. We present the imaging findings of a patient who was diagnosed as isolated left pulmonary artery agenesis. A 27-year-old female patient was admitted to our hospital due to dyspnea during exercise for five years. Chest X-ray revealed minimally small left pulmonary hilum and left lung. She was admitted to our clinic with the suspicion of pulmonary artery pathology. Absent perfusion of the left lung with normal ventilation was visualized on scintigraphy. MDCT angiography of pulmonary arteries showed absent left main pulmonary artery with systemic collaterals around left hemithorax. Pulmonary artery agenesis can be asymptomatic and isolated until adulthood. Both scintigraphy and CT angiography images of pulmonary artery agenesis of a patient are rare in the literature. Pulmonary ventilation- perfusion scintigraphy can be used not only for pulmonary embolism but also pathologies involving pulmonary artery and its branches. (MIRT 2012;21:80-83

  14. Prenatal exposure to antiepileptic drugs and dental agenesis.

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    Pernille E Jacobsen

    Full Text Available OBJECTIVE: The aim of the study was to investigate the association between prenatal exposure to AEDs and the risk of dental agenesis and to differentiate between the possible effects of the different drugs used. METHODS: Data on 214 exposed and 255 unexposed children, aged 12-18 years, were extracted from the Prescription Database of the Central Denmark Region and North Denmark Region and the Danish Medical Birth Registry. The children's dental charts were examined for the presence of dental agenesis. RESULTS: Overall, children exposed to AED in utero had an increased risk of developing dental agenesis, but as a group, the difference was not significant (OR = 1.7; [95% CI: 0.8-3.6]. The risk of developing dental agenesis was three-fold increased (OR = 3.1; [95% CI: 1.3-7.4] in children exposed to valproate in mono- or in poly-therapy with other AEDs than carbamazepine or oxcarbazepine. The risk was further increased (OR = 11.2; [95% CI: 2.4-51.9] in children exposed to valproate and carbamazepine or oxcarbazepine in combination. CONCLUSIONS: The present study shows that dental agenesis is a potential congenital abnormality that is related to prenatal exposure to valproate, and dental agenesis may be considered a sensitive marker for the teratogenicity of valproate.

  15. Waardenburg syndrome with familial unilateral renal agenesis: a new syndrome variant?

    Science.gov (United States)

    Webb, Katie M; Smith, Alisha J; Dansby, Linda M; Diskin, Charles J

    2015-06-01

    A 64-year-old man with Waardenburg syndrome presented with anuria and was subsequently discovered by renal ultrasound to have unilateral renal agenesis. The patient is one of three generations with incidental finding of renal agenesis also marked by the presence of Waardenburg syndrome. To our knowledge, there has been no mention elsewhere in the scientific literature of a variant of Waardenburg syndrome with associated renal agenesis. © 2014 The Authors. Therapeutic Apheresis and Dialysis © 2014 International Society for Apheresis.

  16. Hypohyperdontia: Agenesis of three third molars and mandibular centrals associated with midline supernumerary tooth in mandible

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    Sivakumar Nuvvula

    2010-01-01

    Full Text Available Agenesis of teeth in a patient who also presents with a supernumerary tooth is one of the rare numerical anomalies in human dentition. Agenesis of third molars was shown to be associated with other missing permanent teeth. A review of literature on hypodontia including third molar agenesis, hyperdontia and a concomitant presence of these two conditions which is termed as hypohyperdontia is presented along with a case showing agenesis of three third molars, both mandibular central incisors and a midline supernumerary tooth.

  17. Dental anomalies in an orthodontic patient population with maxillary lateral incisor agenesis.

    Science.gov (United States)

    Citak, Mehmet; Cakici, Elif Bahar; Benkli, Yasin Atakan; Cakici, Fatih; Bektas, Bircan; Buyuk, Suleyman Kutalmış

    2016-01-01

    The purpose of this study was to evaluate the prevalence of dental anomalies in a subpopulation of orthodontic patients with agenesis of maxillary lateral incisors (MLI). The material of the present study included the records of the 1964 orthodontic patients. Panoramic radiographs and dental casts were used to analyze other associated eight dental anomalies, including agenesis of other teeth, dens invaginatus, dens evaginatus, peg shaped MLI, taurodontism, pulp stone, root dilaceration and maxillary canine impaction. Out of the 1964 patients examined, 90 were found to have agenesis of MLI, representing a prevalence of 4.6%. The most commonly found associated-anomalies were agenesis of other teeth (23.3%), peg-shaped MLIs (15.6%), taurodontism (42.2%), and dilacerated teeth (18.9%). Permanent tooth agenesis, taurodontism, peg-shaped maxillary lateral incisor, and root dilacerations are frequently associated with maxillary lateral incisor agenesis.

  18. Agenesia pulmonar unilateral Unilateral pulmonary agenesis

    Directory of Open Access Journals (Sweden)

    Maura Cavada Malcon

    2012-08-01

    Full Text Available A agenesia pulmonar é uma anomalia congênita rara. Relatamos um caso de um menino de 8 anos de idade com agenesia pulmonar à esquerda sem associação com outras malformações. O diagnóstico foi realizado por achados de imagem quando o paciente apresentou sintomas como tosse, sibilância e dispneia sem melhora do quadro clínico após evolução de 30 dias.Pulmonary agenesis is a rare congenital anomaly. We report the case of an 8-year-old boy with left lung agenesis, without any other congenital malformations. When the patient presented symptoms, including cough, wheezing, and dyspnea, with no clinical improvement after a period of 30 days, imaging studies were conducted and the diagnosis was made.

  19. Bilateral multicystic renal dysplasia with potter sequence: A case with penile agenesis

    International Nuclear Information System (INIS)

    Dursun, Ahmet; Ermis, Bahri; Bahadir, Burak; Seckiner, Ilker

    2006-01-01

    Hereditary renal adysplasia (HRA) is rare autosomal dominant condition. Patients have several other anomalies including Potter facies, thoracic, cardiac, and extremity deformities. The case present dysmorphic facial features such as hypertelorism, prominent epicanthic folds, a flat and broad nose, choanal stenosis, low set ears and a receding chin. He had femoral bowing, hypoplastic right tibia and agenesis of the right foot. He had rich and thin skin. He had also a dysplastic empty scrotum, penile agenesis, and anal atresia. The autopsy revealed pulmonary hypoplasia, ventricular septal defect, bilateral multicystic renal dysplasia, agenesis of ureter and bladder, intraabdominal testicles, and a single umbilical artery. The penile agenesis was first reported, and including the consanguinity in the parents might further delineate the bilateral multicystic HRA. Vater/caudal regression anomalies, Mullerian duct/aplasia, unilateral renal agenesis, and cervicothoracic somite anomalies association and Coloboma, heart anomaly, choanal atresia, retardation, retardation, genital and ear anomalies syndrome has been considered in differential diagnosis. (author)

  20. Agenesis of the corpus callosum and autism: a comprehensive comparison.

    Science.gov (United States)

    Paul, Lynn K; Corsello, Christina; Kennedy, Daniel P; Adolphs, Ralph

    2014-06-01

    The corpus callosum, with its ∼200 million axons, remains enigmatic in its contribution to cognition and behaviour. Agenesis of the corpus callosum is a congenital condition in which the corpus callosum fails to develop; such individuals exhibit localized deficits in non-literal language comprehension, humour, theory of mind and social reasoning. These findings together with parent reports suggest that behavioural and cognitive impairments in subjects with callosal agenesis may overlap with the profile of autism spectrum disorders, particularly with respect to impairments in social interaction and communication. To provide a comprehensive test of this hypothesis, we directly compared a group of 26 adults with callosal agenesis to a group of 28 adults with a diagnosis of autism spectrum disorder but no neurological abnormality. All participants had full-scale intelligence quotient scores >78 and groups were matched on age, handedness, and gender ratio. Using the Autism Diagnostic Observation Schedule together with current clinical presentation to assess autistic symptomatology, we found that 8/26 (about a third) of agenesis subjects presented with autism. However, more formal diagnosis additionally involving recollective parent-report measures regarding childhood behaviour showed that only 3/22 met complete formal criteria for an autism spectrum disorder (parent reports were unavailable for four subjects). We found no relationship between intelligence quotient and autism symptomatology in callosal agenesis, nor evidence that the presence of any residual corpus callosum differentiated those who exhibited current autism spectrum symptoms from those who did not. Relative to the autism spectrum comparison group, parent ratings of childhood behaviour indicated children with agenesis were less likely to meet diagnostic criteria for autism, even for those who met autism spectrum criteria as adults, and even though there was no group difference in parent report of current

  1. 4D-MRI analysis of lung tumor motion in patients with hemidiaphragmatic paralysis

    International Nuclear Information System (INIS)

    Dinkel, Julien; Hintze, Christian; Tetzlaff, Ralf; Huber, Peter E.; Herfarth, Klaus; Debus, Juergen; Kauczor, Hans U.; Thieke, Christian

    2009-01-01

    Purpose: To investigate the complex breathing patterns in patients with hemidiaphragmatic paralysis due to malignant infiltration using four-dimensional magnetic resonance imaging (4D-MRI). Patients and methods: Seven patients with bronchial carcinoma infiltrating the phrenic nerve were examined using 1.5 T MRI. The motion of the tumor and of both hemi-diaphragms were measured on dynamic 2D TrueFISP and 4D FLASH MRI sequences. Results: For each patient, 3-6 breathing cycles were recorded. The respiratory-induced mean cranio-caudal displacement of the tumor was 6.6 mm (±2.8 SD). The mean displacement anterior-posterior was 7.4 mm (±2.6), while right-left movement was about 7.4 mm (±4.5). The mediastinum moved sidewards during inspiration, realizing a 'mediastinal shift'. The paralyzed hemidiaphragm and the tumor showed a paradox motion during respiration in five patients. In two patients, the affected hemidiaphragm had a regular, however minimal and asynchronous motion during respiration. Respiratory variability of both tumor and diaphragm motions was about 20% although patients were instructed to breath normally. The findings showed significant differences compared to breathing patterns of patients without diaphragm dysfunction. Conclusion: 4D-MRI is a promising tool to analyze complex breathing patterns in patients with lung tumors. It should be considered for use in planning of radiotherapy to account for individual tumor motion.

  2. Agenesis of the dorsal pancreas

    Science.gov (United States)

    Schnedl, Wolfgang J; Piswanger-Soelkner, Claudia; Wallner, Sandra J; Krause, Robert; Lipp, Rainer W

    2009-01-01

    During the last 100 years in medical literature, there are only 54 reports, including the report of Pasaoglu et al (World J Gastroenterol 2008; 14: 2915-2916), with clinical descriptions of agenesis of the dorsal pancreas in humans. Agenesis of the dorsal pancreas, a rare congenital pancreatic malformation, is associated with some other medical conditions such as hyperglycemia, abdominal pain, pancreatitis and a few other diseases. In approximately 50% of reported patients with this congenital malformation, hyperglycemia was demonstrated. Evaluation of hyperglycemia and diabetes mellitus in all patients with agenesis of the dorsal pancreas including description of fasting blood glucose, oral glucose tolerance test, glycated hemoglobin and medical treatment would be a future goal. Since autosomal dominant transmission has been suggested in single families, more family studies including imaging technologies with demonstration of the pancreatic duct system are needed for evaluation of this disease. With this letter to the editor, we aim to increase available information for the better understanding of this rare disease. PMID:19140241

  3. Bicornuate unicollis uterus with left renal agenesis | Adeyinka ...

    African Journals Online (AJOL)

    This is a case report of a 30 year old lady with 8 years history of 2 Infertility. Radiological findings by Hystero-salpingography (HSG) revealed a Bicornuate Unicolis uterus. This was further confirmed by Ultrasound, which also demonstrated a Left Renal agenesis inclusive. Radiological features, clinical presentation and ...

  4. Bilateral agenesis of the anterior cruciate ligament: MRI evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Bedoya, Maria A.; Jaramillo, Diego [The Children' s Hospital of Philadelphia, Radiology Department, Philadelphia, PA (United States); McGraw, Michael H. [Hospitalof theUniversityof Pennsylvania, Divisionof Orthopaedics, Philadelphia, PA (United States); Wells, Lawrence [The Children' s Hospital of Philadelphia, Division of Orthopaedics, Philadelphia, PA (United States)

    2014-09-15

    Bilateral agenesis of the anterior cruciate ligament (ACL) is extremely rare. We describe a 13-year-old girl who presented with bilateral knee pain without history of trauma; she has two family members with knee instability. Magnetic resonance imaging showed bilateral absence of the ACL, and medial posterior horn meniscal tears. Bilateral arthroscopic partial meniscectomy and anterior cruciate ligament reconstruction was performed. (orig.)

  5. Rare and Common Variants Conferring Risk of Tooth Agenesis

    DEFF Research Database (Denmark)

    Jonsson, L; Magnusson, T E; Thordarson, A

    2018-01-01

    We present association results from a large genome-wide association study of tooth agenesis (TA) as well as selective TA, including 1,944 subjects with congenitally missing teeth, excluding third molars, and 338,554 controls, all of European ancestry. We also tested the association of previously ...

  6. Mutations in PTF1A cause pancreatic and cerebellar agenesis

    NARCIS (Netherlands)

    Sellick, GS; Barker, KT; Stolte-Dijkstra, [No Value; Fleischmann, C; Coleman, RJ; Garrett, C; Gloyn, AL; Edghill, EL; Hattersley, AT; Wellauer, PK; Goodwin, G; Houlston, RS

    2004-01-01

    Individuals with permanent neonatal diabetes mellitus usually present within the first three months of life and require insulin treatment(1,2). We recently identified a locus on chromosome 10p13-p12.1 involved in permanent neonatal diabetes mellitus associated with pancreatic and cerebellar agenesis

  7. Congenital lumbar vertebrae agenesis in a lamb.

    Science.gov (United States)

    Farajli Abbasi, Mohammad; Shojaei, Bahador; Azari, Omid

    2017-01-01

    Congenital agenesis of lumbar vertebrae was diagnosed in a day-old female lamb based on radiology and clinical examinations. There was no neurological deficit in hindlimb and forelimb associated with standing disability. Radiography of the abdominal region revealed absence of lumbar vertebrae. Necropsy confirmed clinical and radiographic results. No other anomaly or agenesis was seen macroscopically in the abdominal and thoracic regions as well as vertebral column. Partial absence of vertebral column has been reported in human and different animal species, as an independent occurrence or associated with other organs anomalies. The latter has been designated as caudal regression syndrome. Vertebral agenesis may arise from irregularity in the differentiation of somites to the sclerotome or sclerotome to the vertebral primordium. Most of the previously reported cases of agenesis were related to the lumbosacral region, lonely or along with other visceral absences. This case was the first report of congenital agenesis of lumbar vertebrae in a lamb.

  8. Middle fossa arachnoid cyst with temporal lobe agenesis accompanying isodense subdural hematoma -a case report-

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung Soo; Choi, Hyung Sik; Kim, Myung Joon; Yang, Seoung Oh; Kim, Chang Jin [Capital Armed Forces General Hospital, Seongnam (Korea, Republic of)

    1987-12-15

    Cysts overlying the temporal lobes have been well described in literature. These are often associated with agenesis of the temporal lobes, and of major neurosurgical interest due to their frequent association with subdural hematoma, a combination that is rarely seen with cysts in other regions. Full features of plain, angiographic, and CT findings of arachnoid cyst with temporal lobe agenesis accompanying isodense subdural hematoma are presented, being very rare in radiologic literature.

  9. Heterotaxy syndrome with associated agenesis of dorsal pancreas and polysplenia: A case report

    Directory of Open Access Journals (Sweden)

    Syed Althaf Ali1

    2015-01-01

    Full Text Available Heterotaxy syndrome is a rare embryological disorder comprising of polysplenia, partial agenesis of dorsal pancreas, malrotation of gut, cardiac and vascular anomalies resulting from failure of development of the usual left–right asymmetry of organs. We report a rare case of heterotaxy syndrome with polysplenia, partial agenesis of dorsal pancreas and malrotation of gut in a 28 year female presenting with subacute intestinal obstruction along with imaging illustrations, brief discussion and thorough review of literature.

  10. Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report

    Directory of Open Access Journals (Sweden)

    Blesneac Cristina

    2016-06-01

    Full Text Available Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH, that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complications in this disease.

  11. Pulmonary Artery Agenesis: A Case Series

    Directory of Open Access Journals (Sweden)

    Meltem Ağca

    2015-04-01

    Full Text Available Pulmonary artery agenesis is a rare congenital abnormality in which atresia was encountered in the short segment of the right or left pulmonary arteries. It can be isolated or associated with cardiac abnormalities such as tetralogy of Fallot, septal defects or pulmonary stenosis.The majority of cases are diagnosed in childhood whereas some cases yield no symptoms until adulthood. We evaluated retrospectively 5 pulmonary artery agenesis cases diagnosed in our clinics between 1998-2010 with respect to the literature.

  12. Agenesia pulmonar unilateral Unilateral pulmonary agenesis

    OpenAIRE

    Maura Cavada Malcon; Claudio Mattar Malcon; Marina Neves Cavada; Paulo Eduardo Macedo Caruso; Lara Flório Real

    2012-01-01

    A agenesia pulmonar é uma anomalia congênita rara. Relatamos um caso de um menino de 8 anos de idade com agenesia pulmonar à esquerda sem associação com outras malformações. O diagnóstico foi realizado por achados de imagem quando o paciente apresentou sintomas como tosse, sibilância e dispneia sem melhora do quadro clínico após evolução de 30 dias.Pulmonary agenesis is a rare congenital anomaly. We report the case of an 8-year-old boy with left lung agenesis, without any other congenital mal...

  13. MR diagnosis of penile agenesis: is it just absence of a phallus?

    Energy Technology Data Exchange (ETDEWEB)

    Goenka, Ajit; Jain, Vaibhav; Sharma, Raju; Gupta, Arun K. [All India Institute of Medical Sciences, Department of Radiodiagnosis, New Delhi (India); Bajpai, Minu [All India Institute of Medical Sciences, Department of Paediatric Surgery, New Delhi (India)

    2008-10-15

    Penile agenesis is an extremely rare anomaly that results from absence of the genital tubercle or its failure to develop into a penis during embryonic life. Associated anomalies of the genitourinary and distal gastrointestinal tracts are frequently present. Imaging modalities, particularly MRI and cystography, play a crucial role in establishing the diagnosis and guiding further management. We report a child with penile agenesis with a urethroanal fistula and a blind-ending anterior urethra who had been reared as a male until presentation. (orig.)

  14. Multidetector-row CT of right hemidiaphragmatic rupture caused by blunt trauma: a review of 12 cases

    International Nuclear Information System (INIS)

    Rees, O.; Mirvis, S.E.; Shanmuganathan, K.

    2005-01-01

    AIM: To determine the usefulness of multidetector-row CT (MDCT) with multiplanar reformatted (MPR) images in the sagittal and coronal plane in diagnosing acute right hemidiaphragmatic rupture. MATERIALS AND METHODS: Twelve patients were identified who received chest and abdominal MDCT after major blunt trauma diagnosed with right diaphragmatic injury. Sagittal and coronal reformations were performed in all cases. The images were retrospectively reviewed by two experienced radiologists for signs of right diaphragm injury, such as direct diaphragm discontinuity, the 'collar sign', the 'dependent viscera sign', and intra-thoracic location of herniated abdominal contents. RESULTS: Of the 12 cases of right hemidiaphragm rupture, diaphragm discontinuity was seen in seven (58%) cases, the collar sign in five (42%), the dependent viscera sign in four (33%), and transdiaphragmatic herniation of the right colon and fat in another. Two variants of the collar sign were apparent on high-quality sagittal and coronal reformations. The first, termed the 'hump sign', describes a rounded portion of liver herniating through the diaphragm forming a hump-shaped mass, and the second, termed the 'band sign,' is a linear lucency across the liver along the torn edges of the hemidiaphragm. The hump sign occurred in 10 (83%) patients and the band sign in four (33%). CONCLUSION: MDCT is very useful in the diagnosis of right hemidiaphragm injury caused by blunt trauma when sagittal and coronal reformatted images are obtained, and should allow more frequent preoperative diagnosis

  15. Chronic renal failure due to unilateral renal agenesis and total renal dysplasia (=aplasia)

    International Nuclear Information System (INIS)

    Kroepelin, T.; Ziupa, J.; Wimmer, B.

    1983-01-01

    Three adult patients with unilateral renal agenesis/total dysplasia (= aplasia) and with an early chronic renal failure are presented. One patient had renal agenesis without ureter bud and ureteric ostium on one side, and reflux pyelonephritis on the other; one had small compact total renal dysplasia (= aplasia) on one side, while chronic uric acid nephropathy (chronic renal disease as a cause of gout) was diagnosed on the other; the third patient had a total large multicystic dysplasia on one side, and on the other a segmental large multicystic dysplasia. Radiological steps and radiodiagnostic criteria are discussed and the combination of urogenital and extraurogenital anomalies is referred to. (orig.)

  16. Does asymptomatic septal agenesis exist? A review of 34 cases

    Energy Technology Data Exchange (ETDEWEB)

    Belhocine, Ouardia; Andre, Christine; Kalifa, Gabriel; Adamsbaum, Catherine [St Vincent de Paul Hospital, Radiology Department, Paris (France)

    2005-04-01

    Primary septal agenesis (PSA) is a rare brain malformation that can be isolated or part of developmental brain abnormalities (holoprosencephaly, septo-optic dysplasia or cortical malformation). Such associated malformation can be subtle, leading to difficulties in the prenatal management of PSA. Moreover, the neurological prognosis of isolated PSA remains debatable. The aims of the study were to specify the patterns and frequency of brain malformations associated with septal agenesis (SA), to identify the clinical prognosis, and to discuss the aetiology of PSA with the new insights provided by molecular genetics. The study consisted of a 14-year retrospective review of brain MRI in 34 patients having PSA (mean age, 5 years). Chiasm and optic nerves were not evaluated. Post-hydrocephalus SA or incomplete data were excluded. The clinical data were correlated to the MRI patterns. The study disclosed 82.5% associated lesions with MRI (28/34): 11 neuronal migration disorders, 9 holoprosencephalies (HP), 7 pituitary stalk interruptions, 1 corpus callosum partial agenesis; 17.5% (6/34) of cases were apparently isolated PAS. Clinically, the patients had motor dysfunction in 68% (23/34), mental retardation in 65% (22/34), blindness in 24% (8/34), endocrinological defects in 21% (7/34) and epilepsy in 18% (6/34) of cases. Nine percent of patients (3/34) were neurologically normal (including one with scoliosis and two infants younger than 2 years at the last follow-up). Patients with bilateral cortical anomalies and HP (even if mild) had the worst neurological prognosis. A severe motor impairment was present without evidence of hemispheric anomaly in 12% of patients (4/34). Interestingly, the frontal lobes were involved in 90% of cortical anomalies and HP, supporting the malformative aetiology of PSA. PSA rarely appears isolated and severe psychomotor impairment may occur in apparently isolated forms. These unfavourable results should be highlighted and need to be confirmed

  17. Deep venous thrombosis and agenesis of the intrahepatic segment of inferior vena cava

    International Nuclear Information System (INIS)

    Velasco, J.; Fernandez, M.M.; Manzanares, R.; Hernando, A.

    1997-01-01

    We present a case of agenesis of the intrahepatic segment of inferior vena cava (IVC) with drainage through the azygos and hemiazygos systems. The presenting sign was deep venous thrombosis (DVT) in both lower extremities. The different imaging studies led to the diagnosis of both the congenital and acquired venous abnormalities, which are discussed. (Author) 14 refs

  18. Vermian agenesis without posterior fossa cyst

    International Nuclear Information System (INIS)

    Adamsbaum, C.; Moreau, V.; Bulteau, C.; Burstyn, J.; Lair Milan, F.; Kalifa, G.

    1994-01-01

    We report 11 cases of vermian partial agenesis without posterior fossa cyst or hemispheric abnormalities. Characteristic MR signs were: absence of the posterior lobe, hypoplasia of the anterior lobe, a narrow sagittal cleft separating the hemispheres (''buttocks sign'') and fourth ventricle deformity. The main clinical signs were complex oculomotor dysfunction and developmental delay. None of the patients had respiratory symptoms. Consideration is given to the relationship between Joubert syndrome and this entity as well as to embroyological data. (orig.)

  19. Total agenesis of the left pericardium

    Directory of Open Access Journals (Sweden)

    J. Glauco Lobo Fº

    1999-09-01

    Full Text Available This is the report of a 46-year-old patient with the preoperative diagnosis of an atrial septal defect (ASD of the ostium secudum type. After sternectomy, partial agenesis of the left pericardium was diagnosed. It is our opinion that, if the radiographic picture is suggestive of this entity, a clinical search for cardiopulmonary anomalies should be performed, because the majority of these associated anomalies can and should be surgically corrected.

  20. Didelphic uterus with imperforate unilateral vagina and ipsilateral renal agenesis: Magnetic resonance

    International Nuclear Information System (INIS)

    Rodrigo, R.M.; Saez, F.; Astigarraga, E.; Rodriguez, O.

    1993-01-01

    We present two cases of didelphic uterus with the clinical appearance of a pelvic mass produced by the hematometra-hematocolpos retained by an imperforate unilateral hemivagina. In both cases, this anomaly was accompanied by ipsilateral renal agenesis. We basically review the diagnosis of this disorder, focusing on magnetic resonance as the technique of choice. (Author) 10 refs

  1. Agenesis of internal carotid artery associated with congenital anterior hypopituitarism

    International Nuclear Information System (INIS)

    Moon, W.-J.; Porto, L.; Lanfermann, H.; Zanella, F.E.; Weis, R.

    2002-01-01

    We report a rare case of unilateral agenesis of the internal carotid artery in association with congenital anterior hypopituitarism. The collateral circulation is supplied by a transsellar intercavernous anastomotic vessel connecting the internal carotid arteries. These abnormalities are well depicted on MRI and MRA. The agenesis of the internal carotid artery may explain the pathogenesis of some of congenital anterior hypopituitarism. (orig.)

  2. Agenesis of maxillary lateral incisors and associated dental anomalies.

    Science.gov (United States)

    Garib, Daniela Gamba; Alencar, Bárbara Maria; Lauris, José Roberto Pereira; Baccetti, Tiziano

    2010-06-01

    The objectives of this study were to evaluate the prevalence of dental anomalies in patients with agenesis of maxillary lateral incisors and to compare the findings with the prevalence of these anomalies in the general population. A sample of 126 patients, aged 7 to 35 years, with agenesis of at least 1 maxillary lateral incisor was selected. Panoramic and periapical radiographs and dental casts were used to analyze other associated dental anomalies, including agenesis of other permanent teeth, ectopia of unerupted permanent teeth, microdontia of maxillary lateral incisors, and supernumerary teeth. The occurrence of these anomalies was compared with prevalence data previously reported for the general population. Statistical testing was performed with the chi-square test (P <0.05) and the odds ratio. Patients with maxillary lateral incisor agenesis had a significantly increased prevalence rate of permanent tooth agenesis (18.2%), excluding the third molars. The occurrence of third-molar agenesis in a subgroup aged 14 years or older (n = 76) was 35.5%. The frequencies of maxillary second premolar agenesis (10.3%), mandibular second premolar agenesis (7.9%), microdontia of maxillary lateral incisors (38.8%), and distoangulation of mandibular second premolars (3.9%) were significantly increased in our sample compared with the general population. In a subgroup of patients aged 10 years or older (n = 115), the prevalence of palatally displaced canines was elevated (5.2%). The prevalences of mesioangulation of mandibular second molars and supernumerary teeth were not higher in the sample. Permanent tooth agenesis, maxillary lateral incisor microdontia, palatally displaced canines, and distoangulation of mandibular second premolars are frequently associated with maxillary lateral incisor agenesis, providing additional evidence of a genetic interrelationship in the causes of these dental anomalies. 2010 American Association of Orthodontists. Published by Mosby, Inc. All

  3. Position and Eruption of Permanent Maxillary Canines in Cases of Maxillary Lateral Incisor Agenesis in Mixed Dentition.

    Science.gov (United States)

    Lamas, Cátia; Lavall, Asela; Pinho, Teresa

    2018-01-01

    Assess whether the permanent maxillary canine (MC) has a natural tendency to erupt mesially in children with maxillary lateral incisors agenesis (MLIA), compared to children without agenesis. This retrospective, observational, cross-sectional study consisted of children between 5 and 12 years old divided into three groups: the first group with unilateral MLIA, in which an intraindividual analysis was performed, the second group presented bilateral MLIA, and the third group with patients without agenesis. These last two groups were matched for comparison interindividual, being pared by sex and maturation of the MC. The canine position in the horizontal sector showed a clear mesial positioning of the MC on the agenesis side in individuals with unilateral MLIA (group 1) when compared with the counter lateral side; and in individuals with bilateral MLIA (Group 2) compared with control Individuals without agenesis (group 3). Even with the maintenance of this deciduous tooth in the dental arch, the MC keeps its tendency to mesial eruption. There is a greater tendency for mesial angulation of the maxillary canine in patients with MLIA, regardless of the presence or absence of deciduous lateral incisor.

  4. Agenesis of the posterior arch of the atlas

    Directory of Open Access Journals (Sweden)

    Torriani Martin

    2002-01-01

    Full Text Available PURPOSE: To illustrate the radiological findings and review the current literature concerning a rare congenital abnormality of the posterior arch of the atlas. CASE REPORT: An adult female without neurological symptoms presented with an absent posterior arch of the atlas, examined with plain films and helical computerized tomography. Complete agenesis of the posterior arch of the atlas is a rare entity that can be easily identified by means of plain films. Although it is generally asymptomatic, atlantoaxial instability and neurological deficits may occur because of structural instability. Computerized tomography provides a means of assessing the extent of this abnormality and can help evaluate the integrity of neural structures. Although considered to be rare entities, defects of the posterior arch of the atlas may be discovered as incidental asymptomatic findings in routine cervical radiographs. Familiarity with this abnormality may aid medical professionals in the correct management of these cases.

  5. [Multiple agenesis and prosthetic restoration].

    Science.gov (United States)

    Renault, P

    1990-03-01

    Cases of multiple agenesia present some difficulties in the treatment planing. Three situations may be encountered: limited agenesia, restored by a fixed, bonded or cemented prosthesis, multiple uni- or bimaxillary agenesia without remaining of deciduous teeth, restored by a fixed, bonded or cemented prosthesis or the partial adjacent prosthesis, multiple uni- or bimaxillary agenesia with remaining of deciduous teeth, restored by means of a supra-dental prosthesis. The first two situations have been described in dental literature and are relatively easy to treat. The same is not true for the third situation, where the decision to keep the temporary teeth considerably increases the difficulty of prosthetic restoration. This subject will be illustrated by the presentation of a clinical case of multiple bi-maxillary agenesia. The patient has: on the maxilla: an absence of 9 permanent teeth (18, 15, 14, 12, 22, 23, 24, 25, 28) and the presence of 4 deciduous teeth (62, 63, 64, 65), on the mandible: an absence of all permanent teeth, with the exception of 36 and 46, and the remaining of 4 deciduous teeth (75, 73, 83, 84). The remaining of deciduous teeth and the presence of a very high inter-arch space led to opting for dental coverage so as to keep the deciduous teeth and a proper vertical dimension. The patient wished to solve his "problem" in the maxilla first, and is not wanting to undergo the extraction of his deciduous teeth. The following therapeutic proposal was adapted: On the maxilla, a three-step procedure: first step: building of metal copings on 13, 16 and 26 and metal-ceramic crowns on 11 and 21, second step: building of telescop crowns on 16 and 26 and clasps on 13, 11 and 21, third step: casting of the removable partial denture framework and soldering to the telescop crowns and clasps. On the mandible, a provisional restoration using a supra-dental resin removable partial denture with ceramic occlusal surfaces was adopted. The aesthetic and functional

  6. Herlyn-Werner-Wunderlich Syndrome Consisting of Uterine Didelphys, Obstructed Hemivagina and Ipsilateral Renal Agenesis in a Newborn

    Directory of Open Access Journals (Sweden)

    Tsung-Hsin Wu

    2012-02-01

    Full Text Available Herlyn-Werner-Wunderlich (HWW syndrome is a rare variant of Müllerian duct anomalies consisting of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome are usually asymptomatic until menarche, when they present with acute lower abdominal pain. Here we report a case of a female newborn with right renal agenesis diagnosed during the pregnancy. The patient presented with a protruding mass over the vaginal introitus that was associated with an obstructed hemivagina and uterine didelphys.

  7. Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases

    Directory of Open Access Journals (Sweden)

    V Govindaraj

    2017-01-01

    Full Text Available Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.

  8. Treatment of cervical agenesis with minimally invasive therapy: Case report

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    Azami Denas Azinar

    2017-11-01

    Full Text Available Cervical agenesis is very rare congenital disorder case with cervical not formed. Because of cervical clogged so that menstruation can not be drained. We Report the case of a19 years old women still single with endometrioma, hematometra, cervical agenesis and perform surgery combination laparoscopy and transvaginally with laparoscopic cystectomy, neocervix, and use catheter no 24f in the new cervix. And now she can currently be normal menstruation. Minimally invasive theraphy of congenital anomalies case is recommended to save reproductive function. Keywords: Cervical agenesis, minimal invasive theraphy

  9. Unilateral Complete Agenesis of Mesonephric Duct Derivatives in an 82-year-Old Male Cadaver: Embryology, Anatomy and Clinical Considerations

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    David G. Darcy

    2017-11-01

    Full Text Available Development of urogenital anatomy in the human fetus is the result of a complex interplay between multiple different tissues. The time course of development is well documented and the morphologic outcomes of insults at various time points during development are predictable. We present a cadaveric case of unilateral agenesis of the left kidney, ureter, bladder hemitrigone, ureteric opening, seminal vesicle, vas deferens, and epididymis. Failure of development of the mesonephric duct early during embryogenesis, likely between the third and fifth week, caused ipsilateral urogenital organ agenesis.

  10. A cross-sectional analysis of the prevalence of tooth agenesis and structural dental anomalies in association with cleft type in non-syndromic oral cleft patients.

    Science.gov (United States)

    Konstantonis, Dimitrios; Alexandropoulos, Alexandros; Konstantoni, Nikoleta; Nassika, Maria

    2017-12-01

    The aim of this study was to investigate the prevalence of tooth agenesis, microdontia, and tooth malformation among non-syndromic oral cleft patients and their potential association with cleft type and gender. Intraoral records and radiographs of 154 patients (97 males and 57 females) were examined. The variables assessed were tooth agenesis, microdontia, dental malformations, and cleft types. The statistics included chi-square and Fisher's exact tests as well as logistic regression to assess any mutual effects of gender and cleft type on the dental variables. Tooth agenesis occurred in 50% of the sample and microdontia in 18%. Non-statistically significant odds ratios for the association of gender and cleft type with tooth agenesis were obtained. Tooth agenesis was substantially higher at the unilateral right CL + P and the bilateral CL + P in quadrant 1 and at the unilateral left CL + P and bilateral CL + P in quadrant 2. It was also higher, at the isolated cleft palate (CP) in quadrants 3 and 4. These results were attributed to teeth 22 (31.8%) and 12 (21.6%) in the maxilla and to teeth 35 (6.1%) and 45 (5.4%) in the mandible. In unilateral CL + P patients, the cleft quadrant that presented tooth agenesis was associated with the side of the cleft. Interdisciplinary treatment of the oral cleft patients should take into consideration the high prevalence of tooth agenesis and their association with the different cleft types. The most frequently affected teeth by cleft are by far the upper lateral incisors. Results indicate that tooth agenesis appears to be a genetically controlled anomaly related to the orofacial cleft development through various genetic links and not caused by the cleft disruptive process.

  11. Learning and memory in individuals with agenesis of the corpus callosum

    OpenAIRE

    Paul, Lynn K.; Erickson, Roger L.; Hartman, Jo Ann; Brown, Warren S.

    2016-01-01

    Damage to long white matter pathways in the cerebral cortex is known to affect memory capacity. However, the specific contribution of interhemispheric connectivity in memory functioning is only beginning to become understood. The present study examined verbal and visual memory processing in individuals with agenesis of the corpus callosum (AgCC) using the Wechsler Memory Scale-Third Edition (WMS-III; Wechsler, 1997b). Thirty participants with AgCC (FSIQ >78) were compared against 30 healthy a...

  12. Radiographic Assessment of Dental Maturation in Children With Dental Agenesis.

    Science.gov (United States)

    Medina, Aida Carolina; Pozo, Rodrigo Del; de Cedres, Lucila Blanco

    Dental agenesis is the most common developmental anomaly in humans, frequently associated with disorders in dental development and maturation. The purpose of this study is to determine radiographic variations in dental maturation in a group of Venezuelan children with dental agenesis. 1,188 panoramic radiographs, from healthy patients ages 5 to 12 years old were studied for agenesis of permanent teeth. Dental maturation was assessed by relative eruption and dental age according to Nolla, comparing children affected with dental agenesis to a stratified control group selected from the same population, excluding children with premature loss of primary teeth in the left quadrants and unclear radiographs. Descriptive analysis, and differences between means and medians (Student t test, Kruskall-Wallis p=0.05) were performed. Medians for Nolla stages were similar between groups, with delay in tooth formation in the agenesis group for second molars (p<0.05) and maxillary lateral incisors and second premolars. Dental age was significantly underestimated for both groups, -0.89 (±0.78) for the control group and -1.20 (±0.95) for the study group. Tooth eruption was similar between groups. Dental age was significantly delayed in Venezuelan children with dental agenesis, with variable significance for tooth formation of studied teeth.

  13. Uterine conserving surgery in a case of cervicovaginal agenesis with unicornuate uterus

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    Vineet Mishra

    2016-01-01

    Full Text Available The presence of cervicovaginal agenesis with unicornuate uterus is a very rare mullerian anomaly. Its true incidence is still unknown. The presence of functioning unicornuate uterus poses a great challenge for a gynecologist because a successful repair could restore normal menses and may preserve a patient′s fertility. Hence, we report a case of 16-year-old unmarried female who presented with chief complaints of primary amenorrhea with cyclical lower abdominal pain. On clinical and radiological evaluation, she was diagnosed with complete cervicovaginal agenesis with right unicornuate uterus (hematometra and hematosalpinx. She underwent vaginoplasty (McIndoes method along with uterovaginal anastomosis by neocervix formation, in order to preserve her uterus. On follow-up, her vagina was completely healed, and she was menstruating normally.

  14. Congenital scoliosis associated with agenesis of the uterine cervix. Case report

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    Monica Matteo

    2004-06-01

    Full Text Available Abstract Background Alterations in the normal sequence of development of müllerian ducts lead to a wide spectrum of reproductive tract abnormalities. A rare form of lack of development, regarding a short tract of the müllerian ducts, leads to the isolated agenesis of the uterine cervix. Anomalies identified among patients with müllerian agenesis include skeletal deformities (i.e., scoliosis of the spine and Klippel-Feil anomaly. Case presentation A 46 years old woman presenting cyphoscoliosis and very low stature (120 cm – 3,93 feet, came to our observation for acute pelvic pain; she also reported primary amenorrhoea associated with cyclic pelvic pain. Clinical and imaging evaluation, evidenced a blind vaginal duct of normal length, left cystic adnexal mass, and enlarged uterus with hematometra. FSH, LH, 17β estradiol and CA-125, karyotype and radiographic study of limbs and vertebral column were also evaluated. At laparotomy, a left ovarian cyst was found. Uterus ended at the isthmus; under this level a thin fibrous tissue band was found, joining the uterus to the vagina. Uterine cervix was replaced by fibrous tissue containing some dilated glands lined with müllerian epithelium. Karyotype resulted 46, XX. The described skeletal deformity, were consistent with Klippel-Feil syndrome. Conclusion We report a case of congenital scoliosis associated with müllerian agenesis limited to uterine cervix, association thus far seen only among patients with Mayer-Rokitansky-Kuster-Hauser syndrome (utero-vaginal agenesis. This case report supports the necessity to evaluate, for accompanying müllerian anomalies, all cases of congenital structural scoliosis in view of the possibility for many müllerian development abnormalities, if timely diagnosed, to be surgically corrected.

  15. Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases.

    Science.gov (United States)

    Fedele, L; Motta, F; Frontino, G; Restelli, E; Bianchi, S

    2013-06-01

    What are the anatomic variants (and their frequencies) of double uterus, obstructed hemivagina and ipsilateral renal agenesis? Most cases examined (72.4%) were of the classic anatomic variant of the Herlyn-Werner-Wunderlich syndrome (with didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis) but the 27.6% of cases are of a rare variant of the syndrome (with uterus septum or cervical agenesis), showing relevant clinical and surgical implications. The extreme variability of anatomic structures involved in this syndrome (both uterus, cervico-vaginal and renal anomalies) is well known, even if a complete and uniform analysis of all its heterogeneous presentations in a large series is lacking. This is a retrospective study with 87 patients referred to our third level referral center between 1981 and 2011. We analyzed the laparoscopic and chart records of 87 women, who referred to our institute with double uterus, unilateral cervico-vaginal obstruction and ipsilateral renal anomalies. Sixty-three of 87 patients had the more classic variant of didelphys uterus with obstructed hemivagina; 10/87 patients had septate bicollis uterus with obstructed hemivagina; 9/87 patients had bicornuate bicollis uterus with obstructed hemivagina; 4/87 patients had didelphys uterus with unilateral cervical atresia; 1/87 patients had bicornuate uterus with one septate cervix and unilateral obstructed hemivagina. This is a retrospective study with a long enrolling period (30 years). New insights in the anatomic variants of this rare syndrome with their relevant surgical implications.

  16. Effect of Vestibulo-Proprioceptive Stimulations in a Child with Agenesis of the Corpus Callosum

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    Hamid Dalvand

    2010-06-01

    Full Text Available Background and Aim: The purpose of the present study was to investigate the effect of vestibulo-proprioceptive stimulations of sensory integration theory on the development of gross and fine motor, language and personal-social functions in a child with agenesis of the corpus callosum.Case: We report a 10.5 month old boy with agenesis of the corpus callosum. The intervention was administered based on sensory integration theory an hour a week for 20 weeks. The exercise intervention consisted of proprioceptive and linear, sustained and low frequency vestibular stimulations on suspension device and physio roll. A Denver Developmental Screening- II and milestones skill testing was completed pre-intervention and monthly. Post-intervention, age of gross motor, fine motor adaptive, language, and personal-social functions significantly improved. Based on milestones skills, maintenance of gross motor functions (e.g. sitting and quadruped position improved. The child could roll from side to side and released objects voluntarily. The reaction time to auditory stimulations became less than 2 seconds.Conclusion: vestibulo-proprioceptive stimulations using the neuroplasticity ability of the central nervous system is effective for development of gross and fine motor, language, and personal-social functions. These exercises can be administered for a child with agenesis of the corpus callosum.

  17. Vaginal agenesis: Experience with sigmoid colon neovaginoplasty

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    Rawat Jiledar

    2010-01-01

    Full Text Available Aim: Objective of this study is to report our experience with sigmoid vaginoplasty in adolescents. Materials and Methods: A retrospective study of children with vaginal atresia and Mayer-Rokitansky-Kuster-Hauser syndrome. The sigmoid segment was used for vaginoplasty in all the cases. Results: Eight children were studied over a period of 7 years. The postoperative complications were ileus in 2, mucosal prolapse of the neovagina in 1, and minor wound infection in 1 patient. Seven patients are on regular follow-up. All the neovaginas were patent and functional. One patient had unacceptable perineal appearance, that is, badly scarred perineum as a late complication. None of the patients had vaginal stenosis or excessive mucus discharge, during follow-up visits. Out of the 7 patients, 2 patients are sexually active and satisfied. Conclusions: Sigmoid vaginoplasty is a safe and acceptable procedure for vaginal agenesis with good cosmetic results and acceptable complications rate. Sigmoid colon vaginoplasty is the treatment of choice because of its large lumen, thick walls resistant to trauma, adequate secretion allowing lubrication, not necessitating prolonged dilatation, and short recovery time.

  18. Müllerian agenesis with hypohidrotic ectodermal dysplasia syndrome.

    Science.gov (United States)

    Whaley, Katie; Winter, Jordan; Eyster, Kathleen M; Hansen, Keith A

    2012-04-01

    To describe the association of müllerian agenesis with hypohidrotic ectodermal dysplasia. Case report. University medical center. A 17-year-old woman with hypohidrotic ectodermal dysplasia referred for evaluation of primary amenorrhea. History, physical examination, and ultrasound. Physical findings of these two syndromes. Physical examination and ultrasound demonstrated müllerian agenesis with findings of hypohidrotic ectodermal dysplasia. This is the first description of the association of müllerian agenesis with ectodermal dysplasia. This rare case might provide further insight into the development of the uterus and the ectoderm as well as its derivatives. Copyright © 2012 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

  19. Agenesis of mandibular second premolar in patient with dental bimaxillary protrusion

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    Carlos Alberto Estevanell Tavares

    Full Text Available ABSTRACT The present study reports the treatment carried out in a patient with mandibular second premolar agenesis associated with early loss of a deciduous second molar, deep overbite, severe overjet and dentoalveolar bimaxillary protrusion, which led to lip incompetence and a convex facial profile. The main objectives of this treatment were: to eliminate the spaces in mandibular arch, correct overbite, as well as eliminate bimaxillary protrusion and lip incompetence, thus leading to a balanced profile. The case was presented to the Brazilian Board of Orthodontics and Dentofacial Orthopedics (BBO as part of the requirements to obtain the title of BBO diplomate.

  20. Gallbladder Agenesis in 17 Dogs: 2006-2016.

    Science.gov (United States)

    Sato, K; Sakai, M; Hayakawa, S; Sakamoto, Y; Kagawa, Y; Kutara, K; Teshima, K; Asano, K; Watari, T

    2018-01-01

    Gallbladder agenesis (GBA) is extremely rare in dogs. To describe the history, clinical signs, diagnosis, treatment, and outcomes of dogs with GBA. Seventeen client-owned dogs with GBA. Medical records from 2006 through 2016 were retrospectively reviewed. Dogs were included when GBA was suspected on abdominal ultrasonography and confirmed by gross evaluation. Signalment, clinical signs, clinicopathological data, diagnostic imaging, histopathology, treatment, and outcome were recorded. Dogs were of 6 different breeds, and Chihuahuas (10 of 17) were most common. Median age at presentation was 1.9 (range, 0.7-7.4) years. Clinical signs included vomiting (5 of 17), anorexia (2 of 17), ascites (2 of 17), diarrhea (1 of 17), lethargy (1 of 17), and seizures (1 of 17). All dogs had increased serum activity of at least 1 liver enzyme, most commonly alanine aminotransferase (15 of 17). Fifteen dogs underwent computed tomography (CT) cholangiography; common bile duct (CBD) dilatation was confirmed in 12, without evidence of bile duct obstruction. Gross evaluation confirmed malformation of the liver lobes in 14 of 17 dogs and acquired portosystemic collaterals in 5 of 17. Ductal plate malformation was confirmed histologically in 16 of 17 dogs. During follow-up (range, 4-3,379 days), 16 of 17 dogs remained alive. Dogs with GBA exhibit clinicopathological signs of hepatobiliary injury and hepatic histopathological changes consistent with a ductal plate abnormality. Computed tomography cholangiography was superior to ultrasound examination in identifying accompanying nonobstructive CBD distention. Computed tomography cholangiography combined with laparoscopic liver biopsy is the preferable approach to characterize the full disease spectrum accompanying GBA in dogs. Copyright © 2018 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

  1. Tooth agenesis in patients referred to an Irish tertiary care clinic for the developmental dental disorders.

    LENUS (Irish Health Repository)

    Hashem, Atef A

    2010-01-01

    PURPOSE: This study was carried out to determine the prevalence, severity and pattern of hypodontia in Irish patients referred to a tertiary care clinic for developmental dental disorders. MATERIALS AND METHODS: Details of 168 patients with hypodontia referred during the period 2002-2006 were entered in a database designed as a national record. Tooth charting was completed using clinical and radiographic examinations. The age of patients ranged from 7-50 years, with a median age of 20 years (Mean: 21.79; SD: 8.005). RESULTS: Hypodontia referrals constituted 65.5% of the total referrals. Females were more commonly affected than males with a ratio of 1.3:1. The number of referrals reflected the population density in this area; the majority were referrals from the public dental service. Mandibular second premolars were the most commonly missing teeth, followed by maxillary second premolars and maxillary lateral incisors; maxillary central incisors were the least affected. Symmetry of tooth agenesis between the right and left sides was an evident feature. Slightly more teeth were missing on the left side (n = 725) than on the right side (n = 706) and in the maxillary arch (n = 768) as compared to the mandibular arch (n = 663). Some 54% of patients had severe hypodontia with more than six teeth missing; 32% had moderate hypodontia, with four to six teeth missing. The most common pattern of tooth agenesis was four missing teeth. CONCLUSION: Hypodontia was a common presentation in a population referred to this tertiary care clinic. The pattern and distribution of tooth agenesis in Irish patients appears to follow the patterns reported in the literature.

  2.  Uterus Didelphys with Obstructed Right Hemivagina, Ipsilateral Renal Agenesis and Right Pyocolpos: A Case Report

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    Hansa Dhar

    2011-11-01

    Full Text Available  Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA Syndrome is a rare congenital anomaly of the female genital tract. Uterus didelphys occurs when the midline fusion of the mullerian ducts is arrested, either completely or incompletely. Women with didelphic uterus may be asymptomatic and unaware of having a double uterus. They may present with complaints of dysmenorrhoea and dyspareunia. A 25 year old woman attending the infertility clinic at Nizwa regional referral hospital presented with history of dysmenorrhoea and foul vaginal discharge with right cystic pelvic mass. She was diagnosed as a case of double uterus with obstructed right hemivagina and right pyocolpos with ipsilateral renal agenesis after routine ultrasonography in the clinic followed by MRI. Excision of the right vaginal septum with drainage of 200 ml of purulent discharge was performed. She was relieved of her symptoms and conceived promptly after the surgical excision of the partial vaginal septum.

  3. Corpus callosum agenesis: Role of fetal magnetic resonance imaging

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    Achour Radhouane

    2016-05-01

    Full Text Available Corpus callosum agenesis (CCA was evaluated by ultrasound examination and magnetic resonance imaging (MRI with many studies. Ultrasonography was able to suspect CCA by indirect signs but a definitive diagnosis of CCA was achieved in rare cases. MRI was able to diagnose complete CCA in majority of cases. Additional neurological abnormalities including heterotopia, gyration anomaly, asymmetry of the cerebral hemispheres, and Dandy-Walker variant were documented, as well as an ocular anomaly which was described, by MRI examination. Prenatal counseling for fetal agenesis of the corpus callosum is difficult as the prognosis is uncertain. The association with other cerebral abnormalities increases the likelihood of a poor outcome and ultrasonographic assessment of the fetal brain is limited. We found MRI to be a safe and useful additional procedure to complement ultrasonographic diagnosis or suspicion of CCA.

  4. Tooth agenesis: from molecular genetics to molecular dentistry

    Czech Academy of Sciences Publication Activity Database

    Matalová, Eva; Fleischmannová, Jana; Sharpe, P. T.; Tucker, A. S.

    2008-01-01

    Roč. 87, č. 7 (2008), s. 617-623 ISSN 0022-0345 R&D Projects: GA AV ČR KJB500450802; GA MŠk OC B23.001; GA ČR GC524/08/J032 Institutional research plan: CEZ:AV0Z50450515 Keywords : tooth agenesis Subject RIV: FF - HEENT, Dentistry Impact factor: 3.142, year: 2008

  5. A case of total agenesis of the corpus callosum

    International Nuclear Information System (INIS)

    Sakamoto, Masanobu; Takeda, Katsuhiko; Bandou, Mitsuaki; Murayama, Shigeo; Sakuta, Manabu

    1985-01-01

    We have reported a case of agenesis of the corpus callosum, in which NMR-CT revealed a complete defect of it, and have examined the localization of the speech center of this patient. The patient is a right-handed 26-year-old man who has complained of headache on the parietal region. His neurological examination revealed only a mild mental difficulty (IQ 77). X-ray CT showed the lateral ventricles to be separated widely and the posterior horns dilated, which were compatible with the agenesis of the corpus callosum. Further, NMR-CT has revealed a total agenesis of the corpus callosum. NMR-CT seems to be highly useful for the detection of the degree of the callosal defect. We have carried out the intracarotid amobarbital injection (Wada's test) for the determination of the lateralization of cerebral speech dominance. It had been reported by some authors that when it comes to the cerebral speech dominance, acallosal patients had no difference between each hemisphere. However, our results have demonstrated a left sided dominance. (author)

  6. Patterns of third-molar agenesis and associated dental anomalies in an orthodontic population.

    Science.gov (United States)

    Celikoglu, Mevlut; Bayram, Mehmet; Nur, Metin

    2011-12-01

    The aim of this study was to investigate the frequency of dental anomalies in orthodontic patients with different patterns of third-molar agenesis, comparing them with patients without third-molar agenesis. A sample of 374 patients with agenesis of at least 1 third molar was divided into 4 groups according to the third-molar agenesis pattern, and a control group of 98 patients without third-molar agenesis was randomly selected from the patient archives. Panoramic radiographs and cast models were used to determine the associated dental anomalies, such as hypodontia, hyperdontia, impaction, dilaceration, microdontia, ectopic eruption, transposition, and transmigration. The Pearson chi-square and Fisher exact tests were used to determine the differences in the distribution of the associated dental anomalies among the groups. The prevalence of agenesis of other teeth (11.2%, n = 42) was significantly greater in our study sample (groups 1-4) than in the control group (group 5) (4.1%, n = 4; P dental anomalies in patients with agenesis of 3 and 4 third molars compared with the control group. Permanent tooth agenesis, microdontia of maxillary lateral incisors, and total dental anomalies are more frequently associated with agenesis of 4 third molars than with the presence of third molars. Copyright © 2011 American Association of Orthodontists. Published by Mosby, Inc. All rights reserved.

  7. The role of Msx1 and Pax9 in pathogenetic mechanisms of tooth agenesis

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    Yani Corvianindya Rahayu

    2009-09-01

    Full Text Available Background: Tooth agenesis is one of the most common developmental anomalies in human, which one or a few teeth are absent because they have never formed, may cause cosmetic or occlusal harm, while severe agenesis which are relatively rare require clinical attention to support and maintain the dental function. Molecular studies have demonstrated that tooth development is under strict genetic control. Purpose: This article want to review the genetic regulating that are responsible for tooth agenesis especially the role of Msx1 and Pax9 in pathogenetic mechanisms of tooth agenesis. Review: Tooth agenesis is a consequence of a qualitatively or quantitatively impaired function of genetic networks, which regulate tooth development. Mutations in Msx1 and Pax9 genes are dominant for tooth agenesis in humans. The Pax9 gene, which codes for a paired domain-containing transcription factor that plays an essential role in the development of mammal dentition, has been associated with selective tooth agenesis in humans and mice. Conclusion: Reduced amount of functional Msx1 or Pax9 protein in the tooth forming cells is able to cause severe and selective tooth agenesis. There are differences in the frequency of agenesis of specific teeth associated with the defects in Msx1 and defects in Pax9.

  8. Agenesis of the gallbladder with hypoplastic cystic duct diagnosed at laparoscopy.

    Science.gov (United States)

    Kwon, A-Hon; Yanagimoto, Hiroaki; Matsui, Yoichi; Imamura, Atsushi

    2006-08-01

    An 86-year-old man was admitted to our department with complaints of intermittent upper abdominal pain. Ultrasonography of the abdomen showed dilated extrahepatic bile ducts containing stones; however, the gallbladder was not clearly identified. Magnetic resonance cholangiopancreatography showed dilated extrahepatic ducts and choledocholithiasis without gallbladder visualization. The stone extraction was performed with endoscopic sphincterotomy. Three-dimensional images using spiral-computed tomography after intravenous-infusion cholangiography clearly demonstrated an obstruction of the cystic duct. The patient was scheduled for laparoscopic cholecystectomy. At laparoscopy, the gallbladder fossa was not identified on the undersurface of the liver. Despite a thorough examination of the intrahepatic (left-sided within the lesser omentum), retroperitoneal, retrohepatic (within the falciform ligament), retroduodenal, and retropancreatic areas using laparoscopic ultrasonography, the gallbladder was not found. After careful dissection of the hepatoduodenal ligament, the dilated extrahepatic bile duct and a 1-cm length of hypoplastic cystic duct were found. Gallbladder agenesis is usually accompanied by the lack of the cystic duct. The present case is the third report of gallbladder agenesis with a patent or hypoplastic cystic duct.

  9. Deep Venous Thrombosis of the Leg, Associated with Agenesis of the Infrarenal Inferior Vena Cava and Hypoplastic Left Kidney (KILT Syndrome in a 14-Year-Old Child

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    Sakshi Bami

    2015-01-01

    Full Text Available Agenesis of the inferior vena cava (IVC is a rare anomaly which can be identified as incidental finding or can be associated with iliofemoral vein thrombosis. IVC agenesis has a known association with renal anomalies which are mainly confined to the right kidney. We describe a case of a 14-year-old male who presented with left leg swelling and pain. Ultrasonography confirmed the presence of left leg deep vein thrombosis (DVT. No underlying hematologic risk factors were identified. A CT scan was obtained which demonstrated absent infrarenal IVC and extensive thrombosis in the left deep venous system and development of collateral venous flow into the azygous/hemiazygous system, with extension of thrombus into paraspinal collaterals. An additional finding in the patient was an atrophic left kidney and stenosis of an accessory left renal artery. Agenesis of the IVC should be considered in a young patient presenting with lower extremity DVT, especially in patients with no risk factors for thrombosis. As agenesis of the IVC cannot be corrected, one should be aware that there is a lifelong risk of lower extremity DVT.

  10. Prevalence of Third Molar Agenesis: Associated Dental Anomalies in Non-Syndromic 5923 Patients.

    Science.gov (United States)

    Sujon, Mamun Khan; Alam, Mohammad Khursheed; Rahman, Shaifulizan Abdul

    2016-01-01

    The aim of this study was to investigate the prevalence of third molar agenesis and other associated dental anomalies in Bangladeshi population and to investigate the relationship of other dental anomalies with the third molar presence/agenesis. A retrospective study was performed using panoramic radiographs of 5923 patients, who ranged in age from 10 to 50 years. All radiographs were analyzed by Planmeca Romexis® 3.0 software (Planmeca Oy, Helsinki, Finland). Pearson chi-square and one way ANOVA (Post Hoc) test were conducted. The prevalence of third molar agenesis was 38.4%. The frequency of third molar agenesis was significantly higher in females than males (p dental anomalies was 6.5%, among them hypodontia was 3.1%. Prevalence of third molar agenesis varies in different geographic region. Among the other dental anomalies hypodontia was more prevalent.

  11. Prenatal Diagnosis of Bilateral Pulmonary Agenesis: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung A; Cho, Jeong Yeon; Lee, Seung Mi; Jun, Jong Kwan; Kang, Ji Eun; Seo, Jeong Wook [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2010-02-15

    We report a case of bilateral pulmonary agenesis (BPA), which was suspected during a prenatal US examination and diagnosed by fetal magnetic resonance imaging (MRI). BPA is an extremely rare congenital anomaly and, although many fetal structural defects can be detected with a high degree of confidence after introducing high-resolution US, the prenatal diagnosis of BPA remains problematic. Other thoracic abnormalities, such as a congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, and pulmonary sequestration, should be excluded from the list of possible diagnoses before coming to the conclusion of BPA, because BPA is absolutely incompatible with extrauterine life, and an accurate internal diagnosis can prevent a futile intervention from being performed.

  12. Multivariate analysis of factors affecting presence and/or agenesis of third molar tooth.

    Directory of Open Access Journals (Sweden)

    Mohammad Khursheed Alam

    Full Text Available To investigate the presence and/or agenesis of third molar (M3 tooth germs in orthodontics patients in Malaysian Malay and Chinese population and evaluate the relationship between presence and/or agenesis of M3 with different skeletal malocclusion patterns and sagittal maxillomandibular jaw dimensions. Pretreatment records of 300 orthodontic patients (140 males and 160 females, 219 Malaysian Malay and 81 Chinese, average age was 16.27±4.59 were used. Third-molar agenesis was calculated with respect to race, genders, number of missing teeth, jaws, skeletal malocclusion patterns and sagittal maxillomandibular jaw dimensions. The Pearson chi-square test and ANOVA was performed to determine potential differences. Associations between various factors and M3 presence/agenesis groups were assessed using logistic regression analysis. The percentages of subjects with 1 or more M3 agenesis were 30%, 33% and 31% in the Malaysian Malay, Chinese and total population, respectively. Overall prevalence of M3 agenesis in male and female was equal (P>0.05. The frequency of the agenesis of M3s is greater in maxilla as well in the right side (P>0.05. The prevalence of M3 agenesis in those with a Class III and Class II malocclusion was relatively higher in Malaysian Malay and Malaysian Chinese population respectively. Using stepwise regression analyses, significant associations were found between Mx (P<0.05 and ANB (P<0.05 and M3 agenesis. This multivariate analysis suggested that Mx and ANB were significantly correlated with the M3 presence/agenesis.

  13. Uterus bicornis bicollis, imperforate hemivagina and ipsilateral renal agenesis: case report and literature review.

    Science.gov (United States)

    DaCosta, V; Christie, L; Wynter, S; Harriott, J; Frederick, J

    2009-09-01

    We present a case of a patient with Uterus bicornis bicollis, imperforate hemivagina and ipsilateral renal agenesis. This group of congenital malformations is often asymptomatic until puberty, when it presents as cyclic dysmenorrhoea, leucorrhoea or a pelvic mass. Magnetic resonance imaging is becoming the preferred modality for delineation of uterine malformations. When congenital abnormalities of the reproductive tract are encountered, a search should also be made for renal anomalies. Patients with Uterus bicornis bicollis and unilateral imperforate vagina are often seen with pain severe enough to mimic an acute abdomen. It is important to keep this unusual congenital malformation in mind in the differential diagnosis of vaginal discharge, pelvic mass and/or abdominopelvic pain in young women so as to avoid inappropriate surgical procedures.

  14. An Unusual Case of Recurrent Pyocolpos Following Midtrimester Miscarriage Revealed as Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA Syndrome

    Directory of Open Access Journals (Sweden)

    Haldar M

    2014-08-01

    Full Text Available Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA Syndrome is a rare congenital anomaly. It mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos. Sometime it presents in unusual way with prolonged vaginal bleeding and profuse vaginal discharge. Here we report case of an 18 years old married female with OHVIRA syndrome presenting late with symptoms of recurrent pyocolpos following dilatation and evacuation for midtrimester miscarriage that was diagnosed on the basis of MRI and managed by vaginal septotomy.

  15. The Prevalence of Palmaris longus agenesis among the Ghanaian population

    Directory of Open Access Journals (Sweden)

    Osonuga A

    2012-10-01

    Full Text Available Objective: Background: Studies have documented the agenesis of Palmaris longus muscle in different populations but none has included the Ghanaian population. Methods: The study involved 226 subjects (130 females and 96 males who are students of the University of Cape Coast, Cape Coast, Ghana. The presence of the PLM was clinically determined using the Schaeffer’s test. Subjects in which the tendon was not visualized or palpable, two other tests were performed to confirm the absence. Results: The total prevalence of absence of PLM was 3.1%; absence on the left hand was commoner than on the right hand. The frequency of PLM absence was also slightly higher in females than in males representing 1.8% and 1.3% respectively. One female had the PLM absent bilaterally whiles a male subject had a trifid tendon on the right forearm with a bifid on the left. Conclusion: The prevalence of PLM agenesis in the Ghanaian population is lower compared to values coated in standard textbook on surgery. PLM is not diminishing as fast as observed in some population hence it is can be readily used as donor tendon by Ghanaian surgeons.

  16. Bochdalek Hernia with Adult Diaphragmatic Agenesis

    Directory of Open Access Journals (Sweden)

    Erkan Akar

    2013-10-01

    Full Text Available    Diaphragmatic hernia arises from pleuro-peritoneal membranes inability to close pericardioperitoneal membranes. Diaphragmatic defect may be located in esophageal hiatus (hiatal hernia, nearby the hiatus (paraesophageal, retrosternal (Morgagni or posterolateral (Bochdalek. Congenital diaphragmatic hernias (CDH diagnosed after neonatal period are defined as late presenting CDH. This group of patients consist 5-31% of CDHs and lead to diagnostic difficulties. A case of adult type Bochdalek hernia who was admitted to our clinic with respiratory problems and recognized late with the absence of left diaphragm was discussed in the light of clinical and surgical methods.

  17. MURCS Association with Partial Duplication of the Distal Long Chromosome 5 and Unilateral Ovarian Agenesis

    Directory of Open Access Journals (Sweden)

    Anna Dabkowska-Huc

    2013-01-01

    Full Text Available A combination of the congenital abnormalities, Müllerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia, is defined as the MURCS association. Various genetic defects have been described in the MURCS association so far, yet the unambiguous molecular basis of these disorders has not been established. We report the case of an 18-year-old woman who presented with primary amenorrhea, right kidney, Arnold-Chiari malformation, and Klippel-Feil syndrome. In addition, the patient showed the following unusual features: right ovarian and Skenes gland agenesis, cubitus valgus with hyperextension and decreased range of motion at elbows, and facial changes. Moreover, the performed DNA analysis showed interstitial duplication in chromosome 5 (5q35.1. In the duplicated region, there are genes whose function is not well known. It is thought that they have an influence on the early stages of development and their joining in the later period can lead to neoplastic disorders, especially leukemias.

  18. Retrovesical cystic lesions in female patients with unilateral renal agenesis or dysplasia

    International Nuclear Information System (INIS)

    Shimada, Kenji; Matsumoto, Fumi; Matsui, Futosi; Obara, Takasi

    2010-01-01

    The objective of this study was to review our experience with female children who have unilateral renal agenesis or renal dysplasia associated with cystic lesions in the reproductive system. Between 1991 and 2007, we treated 26 patients with unilateral renal agenesis or renal dysplasia associated with pelvic, retrovesical or interlabial cystic lesions. In 16 patients, an abnormality either of the kidney or a cystic lesion was detected during the perinatal period. Another 10 patients presented clinical symptoms, including urinary incontinence in five, urinary tract infection in one, and vaginal discharge in four. Based on clinical features and imaging findings, the patients were divided into four groups: those with Gartner's duct cyst (Group 1, n=9); those with uterovaginal duplication with obstructed hemivagina (Herlyn-Werner-Wunderlich syndrome [HWW]; Group 2, n=3); those with both Gartner's duct cyst and HWW syndrome (Group 3, n=3); and those without definitive diagnosis (Group 4, n=11). In Group 1, leakage completely stopped after nephrectomy in three patients, whereas six patients continued to be incontinent despite the removal of dysgenetic kidneys. In Group 2, after the excision of a vaginal septum, no patient presented urinary incontinence. In patients in Group 3, both the mesonephric remnant and mullerian structures were confirmed on imaging or through endoscopy. Because of the high coincidence of genital and renal anomalies, it is recommended that genital systems in female patients with renal anomalies associated with cystic lesions behind the lower urinary tract be investigated. The best imaging modality to use remains under scrutiny. It is important to follow the patients until the age of puberty. The importance of a long-term follow-up in these patients needs to be emphasized. (author)

  19. MRI findings of type II sacral agenesis: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sang A; Kim, Myung Soon; Kwon, Woo Cheol [Dept. of Radiology, Yonsei University Wonju College of Medicine, Wonju Severance Christian Hospital, Wonju (Korea, Republic of)

    2016-07-15

    Sacral agenesis (or caudal regression syndrome) is a rare congenital anomaly involving various levels of coccygeal, sacral, and even lumbar or lower thoracic vertebral dysgenesis, as well as spinal cord abnormalities. A few cases have been previously reported in Korea, especially based upon MRI findings. We describe a case of a 4-year-old girl with partially bilateral agenesis of the sacrum (type II), and club-shaped (chisel-shaped) spinal cord disruption. We also review MRI findings of sacral agenesis, focused on classification and radiological findings.

  20. Agenesis of the dorsal pancreas: a rare cause of insulin-dependent diabetes without abdominal pain: Case report.

    Science.gov (United States)

    Liang, Kaiyong; Ou, Xiaojuan; Huang, Xukai; Lan, Qunfang

    2018-03-01

    Agenesis of the dorsal pancreas is a very rare condition with an unknown pathology and etiology, although it may be associated with autosomal dominant or X-linked dominant inheritance or retinoic acid and hedgehog signaling pathway alterations. This condition usually manifests with abdominal pain or pancreatitis, although some cases are asymptomatic. Approximately 50% of affected patients with this disorder present with hyperglycemia or various other anomalies. We report the case of a 23-year-old Chinese woman who visited the Department of Endocrinology and Metabolism with insulin-dependent diabetes but no specific symptoms, signs, or other deformities. Severe diabetic retinopathy indicated a long period of hyperglycemia. Agenesis of the dorsal pancreas was observed incidentally during the common diagnosis of diabetes, and the diagnosis was established using magnetic resonance imaging, diffusion-weighted imaging, and magnetic resonance cholangiopancreatography. Following the diagnosis of diabetes, insulin replacement therapy was initiated at a dosage of up to 45 U per day. The patient's blood glucose level was monitored, and the insulin dosage was adjusted accordingly. The patient's blood glucose levels gradually normalized after insulin treatment and were subsequently maintained with intensive insulin therapy. Treatment for diabetic retinopathy was provided by the Ophthalmology Department. Agenesis of the dorsal pancreas should be considered in a young patient diagnosed with diabetes who presents with obvious diabetes-related complications (e.g., renal, retinal, or neurological) inconsistent with the course of the disease or a history of other congenital anomalies. We recommend the routine use of computed tomography or magnetic resonance imaging when examining young patients with diabetes.

  1. Agenesis of dorsal pancreas with eventration of diaphragm and intrapancreatic pseudocyst: a rare entity

    Directory of Open Access Journals (Sweden)

    Poras Chaudhary

    2013-02-01

    Full Text Available A case of acute pancreatitis in a 43-year-old male patient, which was eventually diagnosed as agenesis of dorsal pancreas with eventration of left hemidiaphragm and intrapancreatic pseudocyst is being reported.

  2. Pancreas and gallbladder agenesis in a newborn with semilobar holoprosencephaly, a case report.

    Science.gov (United States)

    Hilbrands, Robert; Keymolen, Kathelijn; Michotte, Alex; Marichal, Miriam; Cools, Filip; Goossens, Anieta; Veld, Peter In't; De Schepper, Jean; Hattersley, Andrew; Heimberg, Harry

    2017-05-19

    Pancreatic agenesis is an extremely rare cause of neonatal diabetes mellitus and has enabled the discovery of several key transcription factors essential for normal pancreas and beta cell development. We report a case of a Caucasian female with complete pancreatic agenesis occurring together with semilobar holoprosencephaly (HPE), a more common brain developmental disorder. Clinical findings were later confirmed by autopsy, which also identified agenesis of the gallbladder. Although the sequences of a selected set of genes related to pancreas agenesis or HPE were wild-type, the patient's phenotype suggests a genetic defect that emerges early in embryonic development of brain, gallbladder and pancreas. Developmental defects of the pancreas and brain can occur together. Identifying the genetic defect may identify a novel key regulator in beta cell development.

  3. Agenesis of premolar associated with submerged primary molar and a supernumerary premolar: An unusual case report

    Directory of Open Access Journals (Sweden)

    S. V. S. G. Nirmala

    2012-01-01

    Full Text Available The combination of submerged primary molar, agenesis of permanent successor with a supernumerary in the same place is very rare. The purpose of this article is to report a case of submerged mandibular left second primary molar with supernumerary tooth in the same region along with agenesis of second premolar in an 11-year-old girl, its possible etiological factors, and a brief discussion on treatment options.

  4. Tooth agenesis and craniofacial morphology in pre-orthodontic children with and without morphological deviations in the upper cervical spine

    DEFF Research Database (Denmark)

    Jasemi, Ashkan; Sonnesen, Liselotte

    2016-01-01

    AIM: To analyze differences in prevalence and pattern of tooth agenesis and craniofacial morphology between non syndromic children with tooth agenesis with and without upper cervical spine morphological deviations and to analyze associations between craniofacial morphology and tooth agenesis...... in the two groups together. METHODS: One hundred and twenty-six pre-orthodontic children with tooth agenesis were divided into two groups with (19 children, mean age 11.9) and without (107 children, mean age 11.4) upper spine morphological deviations. Visual assessment of upper spine morphology...... and measurements of craniofacial morphology were performed on lateral cephalograms. Tooth agenesis was evaluated from orthopantomograms. RESULTS: No significant differences in tooth agenesis and craniofacial morphology were found between children with and without upper spine morphological deviations (2.2 ± 1.6 vs...

  5. Incidence of agenesis of palmaris longus in the Andhra population of India

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    K Devi Sankar

    2011-01-01

    Full Text Available Background : The knowledge of Palmaris longus (PL is a growing interest for its wide role in reconstructive plastic surgeries as a donor tendon for transfer or transplant. The prevalence of the PL agenesis has been well-documented by many authors in different ethnic groups or populations. Many conventional tests for determining the presence of the PL has been described, but lamentably there are many discrepancies in confirming its presence or absence. Slight modifications of the prevailing methods can still give authenticate results. Aim : This prospective study was conducted to determine the incidence of unilateral and bilateral agenesis of PL and its association with sex and side of the limb in the Andhra population of India. Materials and Methods : A total of 942 subjects of both sexes belonging to 18-23 years were used to access the PL using various tendon examination techniques including our modified Schaeffer′s test. The data collected were analyzed by Pearsons χ2 test using SPSS software. Results : Overall agenesis of muscle in both sexes was 264 (28.0%, out of which 40.2% was seen in females and 14.7% in males with the ratio of 3:1. The unilateral agenesis was seen in 70.5% and bilateral agenesis in 29.5% subjects. The left side agenesis was seen in 51.6% and right side in 48.4% subjects. Conclusions : The prevalence of bilateral and unilateral agenesis was more common on left side with a greater likelihood in the female subjects. The proposed technique could bring better results in all subjects and can be implemented in manual examination of PL.

  6. Presentations

    International Nuclear Information System (INIS)

    2007-01-01

    The presented materials consist of presentations of international workshop which held in Warsaw from 4 to 5 October 2007. Main subject of the meeting was progress in manufacturing as well as research program development for neutron detector which is planned to be placed at GANIL laboratory and will be used in nuclear spectroscopy research

  7. Emotional Intelligence in Agenesis of the Corpus Callosum.

    Science.gov (United States)

    Anderson, Luke B; Paul, Lynn K; Brown, Warren S

    2017-05-01

    People with agenesis of the corpus callosum (AgCC) with normal general intelligence have deficits in complex cognitive processing, as well as in social cognition. It is uncertain the extent to which impoverished processing of emotions may contribute to social processing deficiencies. We used the Mayer-Salovey-Caruso Emotional Intelligence Test to clarify the nature of emotional intelligence in 16 adults with AgCC. As hypothesized, persons with AgCC exhibited greater disparities from norms on tests involving more socially complex aspects of emotions. The AgCC group did not differ from norms on the Experiential subscale, but they were significantly below norms on the Strategic subscale. These findings suggest that the corpus callosum is not essential for experiencing and thinking about basic emotions in a "normal" way, but is necessary for more complex processes involving emotions in the context of social interactions. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  8. Presentations

    International Nuclear Information System (INIS)

    2007-01-01

    The PARIS meeting held in Cracow, Poland from 14 to 15 May 2007. The main subjects discussed during this meeting were the status of international project dedicated to gamma spectroscopy research. The scientific research program includes investigations of giant dipole resonance, probe of hot nuclei induced in heavy reactions, Jacobi shape transitions, isospin mixing and nuclear multifragmentation. The mentioned programme needs Rand D development such as new scintillations materials as lanthanum chlorides and bromides as well as new photo detection sensors as avalanche photodiodes - such subjects are also subjects of discussion. Additionally results of computerized simulations of scintillation detectors properties by means of GEANT- 4 code are presented

  9. Presentation

    Directory of Open Access Journals (Sweden)

    Eduardo Vicente

    2013-06-01

    Full Text Available In the present edition of Significação – Scientific Journal for Audiovisual Culture and in the others to follow something new is brought: the presence of thematic dossiers which are to be organized by invited scholars. The appointed subject for the very first one of them was Radio and the invited scholar, Eduardo Vicente, professor at the Graduate Course in Audiovisual and at the Postgraduate Program in Audiovisual Media and Processes of the School of Communication and Arts of the University of São Paulo (ECA-USP. Entitled Radio Beyond Borders the dossier gathers six articles and the intention of reuniting works on the perspectives of usage of such media as much as on the new possibilities of aesthetical experimenting being build up for it, especially considering the new digital technologies and technological convergences. It also intends to present works with original theoretical approach and original reflections able to reset the way we look at what is today already a centennial media. Having broadened the meaning of “beyond borders”, four foreign authors were invited to join the dossier. This is the first time they are being published in this country and so, in all cases, the articles where either written or translated into Portuguese.The dossier begins with “Radio is dead…Long live to the sound”, which is the transcription of a thought provoking lecture given by Armand Balsebre (Autonomous University of Barcelona – one of the most influential authors in the world on the Radio study field. It addresses the challenges such media is to face so that it can become “a new sound media, in the context of a new soundscape or sound-sphere, for the new listeners”. Andrew Dubber (Birmingham City University regarding the challenges posed by a Digital Era argues for a theoretical approach in radio studies which can consider a Media Ecology. The author understands the form and discourse of radio as a negotiation of affordances and

  10. Discordant tooth agenesis and peg-shaped in a pair of monozygotic twins: Clinical and molecular study

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    Lívia Azeredo Alves Antunes

    2013-01-01

    Full Text Available The present report aimed to study an uncommon case of a pair of twins that presented a discordant dental phenotype. Family investigation, clinical, radiographic examination and molecular analysis were performed in both girls. Molecular analysis confirmed the monozygosity by deoxyribonucleic acid chip technology. One twin presented tooth agenesis in left upper lateral incisor and peg-shaped on the contra lateral side while the other twin had no dental alterations. The dental casts study employed digital caliper to compare morphological dimensions and showed alteration only in peg-shaped tooth. In conclusion, this study provide support that one or more mutated gene could cause discordances in dental phenotype in these monozygotic twins.

  11. Proverb comprehension in individuals with agenesis of the corpus callosum.

    Science.gov (United States)

    Rehmel, Jamie L; Brown, Warren S; Paul, Lynn K

    2016-09-01

    Comprehension of non-literal language involves multiple neural systems likely involving callosal connections. We describe proverb comprehension impairments in individuals with isolated agenesis of the corpus callosum (AgCC) and normal-range general intelligence. Experiment 1 compared Gorham Proverb Test (Gorham, 1956) performance in 19 adults with AgCC and 33 neurotypical control participants of similar age, sex, and intelligence. Experiment 2 used the Proverbs subtest of the Delis-Kaplan Executive Function System (D-KEFS, 2001) to compare 19 adults with AgCC and 17 control participants with similar age, sex, and intelligence. Gorham Proverbs performance was impaired in the AgCC group for both the free-response and multiple-choice tasks. On the D-KEFS proverbs test, the AgCC group performed significantly worse on the free-response task (and all derivative scores) despite normal levels of performance on the multiple-choice task. Covarying verbal intelligence did not alter these outcomes. However, covarying a measure of non-literal language comprehension considerably reduced group differences in proverb comprehension on the Gorham test, but had little effect on the D-KEFS group differences. The difference between groups seemed to be greatest when participants had to generate their own interpretation (free response), or in the multiple choice format when the test included many proverbs that were likely to be less familiar. Taken together, the results of this study clearly show that proverb comprehension is diminished in individuals with AgCC compared to their peers. Copyright © 2016 Elsevier Inc. All rights reserved.

  12. A Case of a Newborn with Agenesis of the Corpus Callosum Complicated with Ocular Albinism

    Directory of Open Access Journals (Sweden)

    Michiko Miki

    2016-05-01

    Full Text Available Purpose: To report a case of ocular albinism found in a newborn infant in whom agenesis of the corpus callosum (ACC was indicated in utero. Case Report: This study involved a female newborn who was delivered after a gestational period of 41 weeks. The patient was referred to the Obstetrics Department at Takatsuki Hospital, Takatsuki City, Japan, after the indication of ACC by magnetic resonance imaging (MRI at a nearby clinic during the fetal period. At birth, the baby’s weight was 2,590 g, and ACC and ventricular enlargement were found by cranial sonography and cranial MRI. While initial ophthalmic findings noted partial loss of pigmentation of the iris and hypopigmentation of broad areas of the fundus in both eyes, nystagmus was not observed. The patient’s hair pigment was slightly diluted, and the color of her skin was slightly off-white. At 2 years after birth, obvious mental retardation was observed. With regard to other systemic findings, no apparent heart, kidney, or immune system abnormalities were found. Conclusion: Although the patient in question is presently growing without any major systemic problems, it will be necessary in the future to pay attention to any changes in systemic and ophthalmic findings.

  13. Securing the airway in a child with tracheal agenesis – an alternative perspective

    Directory of Open Access Journals (Sweden)

    Slavomir Nosal

    2011-03-01

    Full Text Available Tracheal agenesis is an extremely rare, typically fatal congenital anomaly characterised by typical clinical manifestation, failed endotracheal intubation and difficult emergency management. The prognosis and possibility of surgical correction rest on early diagnosis, anatomy, birth weight, and associated anomalies. Tracheal agenesis causes considerable resuscitation difficulties immediately after birth, as tracheal intubation is impossible. Although emergency management, by either bag and mask ventilation or oesophageal intubation, provides some pulmonary gas exchange through tracheo-oesophageal connection, they are associated with overdistension of the stomach and the risk of stomach perforation. We have performed selective intubation of the distal trachea in a child with tracheal agenesis through the tracheo-oesophageal connection with the help of a fibroendoscope. A standard guide wire was passed through the working port of the flexible endoscope and under direct vision was directed through the tracheo-oesophageal connection into the distal trachea. At this point, the endoscope was removed, leaving the guide wire in place. The endotracheal tube size 2 Fr was passed over the guide wire into the distal trachea. The infant showed dramatic improvement and maintained good saturations on mechanical ventilation. The infant was taken for a computed tomography (CT scan, which showed Floyd’s type II tracheal agenesis, the oesophagus connecting with the distal trachea before it bifurcates. The endotracheal tube was seen ending in the carina. Transoesophageal intubation of the short distal segment of the trachea through the tracheo-oesophageal connection might offer a new perspective for short-term securing of the airway in a child with tracheal agenesis and should be considered in any child with suspected tracheal agenesis to buy the time while evaluating the exact anatomy of the anomaly and the possibility of tracheal reconstruction in selected

  14. Bilateral Deep Vein Thrombosis Associated with Inferior Vena Cava Agenesis in a Young Patient Manifesting as Low Back Pain

    Directory of Open Access Journals (Sweden)

    Felipe Langer

    2017-04-01

    Full Text Available Congenital absence of the inferior vena cava is a rare vascular anomaly, and most cases are asymptomatic. Nevertheless, patients with inferior vena cava malformations may have increased risk of deep venous thrombosis. Particularly, cases of bilateral deep venous thrombosis may arise owing to an insufficient collateral venous drainage from the lower limbs. We hereby describe a case of a previously healthy young male patient presenting with bilateral lower limb deep venous thrombosis as the initial clinical manifestation of congenital inferior vena cava agenesis. We conclude that in young patients presenting with deep venous thrombosis, especially when thrombosis occurs spontaneously, bilaterally, or recurrently, inferior vena cava anomalies should be thoroughly investigated and ruled out as appropriate.

  15. Unilateral renal agenesis associated with ovarian cysts in a 19 year ...

    African Journals Online (AJOL)

    MJP

    2015-10-28

    379. doi: http://dx.doi.org/10.14194/ijmbr.4.3.2. How to cite this article: Anyabolu E.N,. Chukwuonye I.I, Anyabolu A.E, Enwere O.O.. Unilateral renal agenesis associated with ovarian cysts in a 19 year old woman in Orlu,.

  16. Evidence-based, ethically justified counseling for fetal bilateral renal agenesis

    Science.gov (United States)

    Thomas, Alana N.; McCullough, Laurence B.; Chervenak, Frank A.; Placencia, Frank X.

    2017-01-01

    Background Not much data are available on the natural history of bilateral renal agenesis, as the medical community does not typically offer aggressive obstetric or neonatal care asbilateral renal agenesis has been accepted as a lethal condition. Aim To provide an evidence-based, ethically justified approach to counseling pregnant women about the obstetric management of bilateral renal agenesis. Study design A systematic literature search was performed using multiple databases. We deploy an ethical analysis of the results of the literature search on the basis of the professional responsibility model of obstetric ethics. Results Eighteen articles met the inclusion criteria for review. With the exception of a single case study using serial amnioinfusion, there has been no other case of survival following dialysis and transplantation documented. Liveborn babies die during the neonatal period. Counseling pregnant women about management of pregnancies complicated by bilateral renal agenesis should be guided by beneficence-based judgment informed by evidence about outcomes. Conclusions Based on the ethical analysis of the results from this review, without experimental obstetric intervention, neonatal mortality rates will continue to be 100%. Serial amnioinfusion therefore should not be offered as treatment, but only as approved innovation or research. PMID:28222038

  17. Professional and patient-based evaluation of oral rehabilitation in patients with tooth agenesis

    DEFF Research Database (Denmark)

    Dueled, Erik; Gotfredsen, Klaus; Trab Damsgaard, Mogens

    2009-01-01

    OBJECTIVES: The outcome of oral rehabilitation is usually monitored with clinical tests rather than by patient's perception of change. The aim of this study was to describe the objective measure and subjective perception of oral rehabilitation in patients with tooth agenesis. MATERIAL AND METHODS...

  18. Novel PAX9 gene polymorphisms and mutations and susceptibility to tooth agenesis in the Czech population

    Czech Academy of Sciences Publication Activity Database

    Hloušková, A.; Bonczek, Ondřej; Izakovičová Hollá, L.; Lochman, J.; Šoukalová, J.; Štembírek, Jan; Míšek, Ivan; Černochová, P.; Krejčí, P.; Vaněk, J.; Šerý, Omar

    2015-01-01

    Roč. 36, č. 5 (2015), s. 101-106 ISSN 0172-780X R&D Projects: GA MZd(CZ) NT11420 Institutional support: RVO:67985904 Keywords : odontogenesis * tooth agenesis * PAX9 gene Subject RIV: FF - HEENT, Dentistry Impact factor: 0.946, year: 2015

  19. Aicardi's syndrome: (agenesis of the corpus callosum, infantile spasms, and ocular anomalies).

    Science.gov (United States)

    Dinani, S; Jancar, J

    1984-06-01

    A case of Aicardi's syndrome is reported. A 12-year-old mentally handicapped female has all the characteristics of the syndrome: agenesis of the Corpus callosum; female infant with mental handicap, epilepsy, characteristic eye lesions, vertebral anomalies and abnormal EEG pattern. The results of supporting examination and investigations are noted.

  20. Tooth agenesis in osteogenesis imperfecta related to mutations in the collagen type I genes.

    Science.gov (United States)

    Malmgren, B; Andersson, K; Lindahl, K; Kindmark, A; Grigelioniene, G; Zachariadis, V; Dahllöf, G; Åström, E

    2017-01-01

    Osteogenesis imperfecta (OI) is a heterogeneous group of disorders of connective tissue, mainly caused by mutations in the collagen type I genes (COL1A1 and COL1A2). Tooth agenesis is a common feature of OI. We investigated the association between tooth agenesis and collagen type I mutations in individuals with OI. In this cohort study, 128 unrelated individuals with OI were included. Panoramic radiographs were analyzed regarding dentinogenesis imperfecta (DGI) and congenitally missing teeth. The collagen I genes were sequenced in all individuals, and in 25, multiplex ligation-dependent probe amplification was performed. Mutations in the COL1A1 and COL1A2 genes were found in 104 of 128 individuals. Tooth agenesis was diagnosed in 17% (hypodontia 11%, oligodontia 6%) and was more frequent in those with DGI (P = 0.016), and in those with OI type III, 47%, compared to those with OI types I, 12% (P = 0.003), and IV, 13% (P = 0.017). Seventy-five percent of the individuals with oligodontia (≥6 missing teeth) had qualitative mutations, but there was no association with OI type, gender, or presence of DGI. The prevalence of tooth agenesis is high (17%) in individuals with OI, and OI caused by a qualitative collagen I mutation is associated with oligodontia. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Pancreas-specific deletion of mouse Gata4 and Gata6 causes pancreatic agenesis

    Science.gov (United States)

    Xuan, Shouhong; Borok, Matthew J.; Decker, Kimberly J.; Battle, Michele A.; Duncan, Stephen A.; Hale, Michael A.; Macdonald, Raymond J.; Sussel, Lori

    2012-01-01

    Pancreatic agenesis is a human disorder caused by defects in pancreas development. To date, only a few genes have been linked to pancreatic agenesis in humans, with mutations in pancreatic and duodenal homeobox 1 (PDX1) and pancreas-specific transcription factor 1a (PTF1A) reported in only 5 families with described cases. Recently, mutations in GATA6 have been identified in a large percentage of human cases, and a GATA4 mutant allele has been implicated in a single case. In the mouse, Gata4 and Gata6 are expressed in several endoderm-derived tissues, including the pancreas. To analyze the functions of GATA4 and/or GATA6 during mouse pancreatic development, we generated pancreas-specific deletions of Gata4 and Gata6. Surprisingly, loss of either Gata4 or Gata6 in the pancreas resulted in only mild pancreatic defects, which resolved postnatally. However, simultaneous deletion of both Gata4 and Gata6 in the pancreas caused severe pancreatic agenesis due to disruption of pancreatic progenitor cell proliferation, defects in branching morphogenesis, and a subsequent failure to induce the differentiation of progenitor cells expressing carboxypeptidase A1 (CPA1) and neurogenin 3 (NEUROG3). These studies address the conserved and nonconserved mechanisms underlying GATA4 and GATA6 function during pancreas development and provide a new mouse model to characterize the underlying developmental defects associated with pancreatic agenesis. PMID:23006325

  2. Perawatan Teknik Begg Pada Maloklusi Klas I Dengan Kaninus Impaksi dan Insisivus Lateral Agenesis

    Directory of Open Access Journals (Sweden)

    Kristina Wijaya Gunawan

    2013-06-01

    Full Text Available Impaksi kaninus maksila sering dijumpai pada sisi palatal daripada labial. Agenesis adalah anomali pertumbuhan akibat tidak ada satu atau lebih benih gigi. Laporan kasus ini bertujuan memaparkan kemajuan perawatan kasus Maloklusi klas I dengan kaninus impaksi dan insisivus lateral agenesis menggunakan alat cekat teknik Begg. Seorang pasien usia 20 tahun datang mengeluhkan gigi-gigi depan atas dan bawah yang berjejal sehingga mengganggu penampilan. Perawatan bertujuan untuk koreksi Maloklusi Angle klas I tipe dentoskeletal dengan deepbite, crossbite gigi 25 terhadap 35, pergeseran midline dental maksila dan mandibula ke kanan sebesar 2,5 mm dan 3,0 mm, 13 impaksi vertikal pada sisi labial, 42 agenesis, dan edentulous parsial regio 36. Koreksi dilakukan dengan pencabutan 14, 25, pemanfaatan ruang bekas pencabutan 36 dan exposure gigi kaninus yang impaksi. Tahap pertama teknik Begg adalah leveling, unraveling, dan bite opening, diikuti dengan koreksi midline dan penutupan sisa ruang bekas pencabutan. Kesimpulan: perawataan ortodontik menggunakan teknik Begg yang dilakukan simultan dengan exposure kaninus impaksi labial dengan closed eruption technique dapat memberikan hasil yang memuaskan. Treatment for Class I Malocclusion with Impacted Canine and Agenesis Lateral Incisor Using Begg Technique. Maxillary canine impaction occurs commonly on the palatal than labial side. Agenesis is a developmental anomaly condition because of the absence of one or more tooth buds. This case report aims to explain the treatment progress of class I malocclusion with impacted canine and agenesis lateral incisor using fixed appliance through Begg technique. A 20-year-old female patient complained about her upper and lower anterior dental crowding that disturbed her appearance. The treatment aims to correct the Angle class I malocclusion dentoskeletal types with deepbite, crossbite 25 to 35, maxillary and mandibulary dental midline shift to the right by 2.5 mm and 3.0 mm

  3. Neurodevelopmental outcome in prenatally diagnosed isolated agenesis of the corpus callosum.

    Science.gov (United States)

    Folliot-Le Doussal, Lise; Chadie, Alexandra; Brasseur-Daudruy, Marie; Verspyck, Eric; Saugier-Veber, Pascale; Marret, Stéphane

    2018-01-01

    Neurodevelopmental outcome in children with agenesis of the corpus callosum (ACC) is correlated with the presence or absence of associated brain abnormalities. Indeed, neurodevelopmental outcome shows severe disabilities when the ACC is not isolated whereas in isolated forms, the neurologic development is mainly normal. Contrary to data in several published studies, the prognosis remains uncertain even in isolated forms, which may lead in France to medical termination of pregnancy. To evaluate long-term neurodevelopmental outcome in children with prenatally diagnosed isolated ACC. This is a follow-up study conducted in Normandy (France). It included a cohort of 25 children born between January 1991 and June 2016, with a prenatal diagnosis of isolated ACC and who were followed for at least two years. The average follow-up was 8±5years. ACC was complete in 17 patients (68%), partial in 5 (20%) and hypoplastic in 3 (12%). Whereas global motor development was normal in each case, normal neurodevelopmental outcome or mild disabilities occurred in 88% children and moderate/severe neuro-disabilities were present in 12% of children. Wechsler Intelligence Scale for Children-IV evaluations and Intellectual Total Quotients were within normal range, but we observed lower scores in verbal comprehension, social judgment, executive functions. A lower score in morphosyntax was observed among 52% of children with oral language disorders. Neurodevelopmental outcome was favorable in most of our patients with isolated ACC, but mild learning disabilities emerged in older children. Long-term follow-up until school age is essential to provide early diagnosis and appropriate care support. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. Hereditary motor and sensory neuropathy with agenesis of the corpus callosum.

    Science.gov (United States)

    Dupré, Nicolas; Howard, Heidi C; Mathieu, Jean; Karpati, George; Vanasse, Michel; Bouchard, Jean-Pierre; Carpenter, Stirling; Rouleau, Guy A

    2003-07-01

    Hereditary motor and sensory neuropathy associated with agenesis of the corpus callosum (OMIM 218000) is an autosomal recessive disease of early onset characterized by a delay in developmental milestones, a severe sensory-motor polyneuropathy with areflexia, a variable degree of agenesis of the corpus callosum, amyotrophy, hypotonia, and cognitive impairment. Although this disorder has rarely been reported worldwide, it has a high prevalence in the Saguenay-Lac-St-Jean region of the province of Quebec (Canada) predominantly because of a founder effect. The gene defect responsible for this disorder recently has been identified, and it is a protein-truncating mutation in the SLC12A6 gene, which codes for a cotransporter protein known as KCC3. Herein, we provide the first extensive review of this disorder, covering epidemiological, clinical, and molecular genetic studies.

  5. Dental root agenesis following radiation and antineoplastic therapy: A Case Report

    Directory of Open Access Journals (Sweden)

    Abdul Hafiz

    2016-01-01

    Full Text Available The survival rates of patients suffering from various childhood neoplasms have improved dramatically with the advent of chemo-radiation therapy. The harmful effects of chemo-radiation therapy in the oro-facial region such as root agenesis, short roots, impaired amelogenesis, dentinogenesis, radiation caries, and other soft tissue pathologies are well recognized. In spite of these documented risks, the antineoplastic treatment modalities continue to serve the patient for overall improvement in survival and quality of life. However, a thorough understanding of the growth and development process and its relation with the complex antineoplastic treatment is very important for all clinicians. Such awareness could significantly improve the status of patients in the posttreatment period with the implementation of proper preventive and interceptive measures. This article intends to document a case of root agenesis that developed secondary to chemo-radiation therapy in a 12-year-old girl.

  6. Agenesis of the dorsal pancreas: systematic review of a clinical challenge

    Directory of Open Access Journals (Sweden)

    Javier A. Cienfuegos

    Full Text Available Background: Agenesis of the dorsal pancreas is a rare malformation. Since 1911 and until 2008, 53 cases have been reported. Several authors have recently described the association of this anomaly with neoplasia of the ventral pancreas, thus we performed a systematic review of the literature from 2008 to 2015. Methods: A systematic review of the Medline and ISI Web of Science Databases from 2008 until 2015 was carried out, and 30 articles which met the inclusion criteria were identified that included a total of 53 patients: 7 children and 46 adults. Conclusions: Although dorsal pancreatic agenesis is a rare malformation, given its association with non-alcoholic pancreatitis and neoplasia of the residual pancreas, physicians should maintain an expectant attitude.

  7. Caudal Regression Syndrome with Partial Agenesis of the Corpus callosum and Partial Lobar Holoprosencephaly

    Science.gov (United States)

    Hashami, Hilal Al; Bataclan, Maria F; Mathew, Mariam; Krishnan, Lalitha

    2010-01-01

    Caudal regression syndrome is a rare fetal condition of diabetic pregnancy. Although the exact mechanism is not known, hyperglycaemia during embryogenesis seems to act as a teratogen. Independently, caudal regression syndrome (CRS), agenesis of the corpus callosum (ACC) and partial lobar holoprosencephaly (HPE) have been reported in infants of diabetic mothers. To our knowledge, a combination of all these three conditions has not been reported so far. PMID:21509087

  8. Caudal Regression Syndrome with Partial Agenesis of the Corpus callosum and Partial Lobar Holoprosencephaly: Case report.

    Science.gov (United States)

    Hashami, Hilal Al; Bataclan, Maria F; Mathew, Mariam; Krishnan, Lalitha

    2010-04-01

    Caudal regression syndrome is a rare fetal condition of diabetic pregnancy. Although the exact mechanism is not known, hyperglycaemia during embryogenesis seems to act as a teratogen. Independently, caudal regression syndrome (CRS), agenesis of the corpus callosum (ACC) and partial lobar holoprosencephaly (HPE) have been reported in infants of diabetic mothers. To our knowledge, a combination of all these three conditions has not been reported so far.

  9. Uterus didelphys with partial vaginal septum and distal vaginal agenesis: an unusual anomaly

    International Nuclear Information System (INIS)

    Singhal, S.R.; Lakra, P.; Bishnoi, P.; Rohilla, S.; Dahiya, P.

    2013-01-01

    Complex malformations of female genital tract are not so common. Their correct identification is of paramount importance for appropriate management. A thorough knowledge of embryology, pre-operative imaging with MRI and examination under anaesthesia is essential to identify accurately the constellation of anomalies and to plan appropriate management. This case reports the coexistence of Mullerian abnormality with vaginal agenesis in an 18 years old girl which was managed by dissecting the lower half of vagina and pull-through vaginoplasty. (author)

  10. Genetic epidemiology of tooth agenesis in Japan: a population- and family-based study.

    Science.gov (United States)

    Machida, J; Nishiyama, T; Kishino, H; Yamaguchi, S; Kimura, M; Shibata, A; Tatematsu, T; Kamamoto, M; Yamamoto, K; Makino, S; Miyachi, H; Shimozato, K; Tokita, Y

    2015-08-01

    Tooth agenesis is one of the most common congenital anomalies in humans. However, the etiology of tooth agenesis remains largely unclear, as well as evidence base useful for genetic counseling. Therefore, we estimated the prevalence and sibling recurrence risk, and investigated agenetic patterns systematically. Tooth agenesis was classified into two subtypes: hypodontia (one to five missing teeth) and oligodontia (six or more missing teeth). The prevalence of these two subtypes were 6.8% [95% confidence interval (CI): 6.1-7.7%] and 0.1% (95% CI: 0.04-0.3%), respectively, and sibling recurrence risk of these were 24.5% (95% CI: 13.8-38.3%) and 43.8% (95% CI: 26.4-62.3%), respectively. This result suggests that the severe phenotype, oligodontia, might be mostly transmitted in a dominant fashion. Using a simple statistical modeling approach, our data were found to be consistent with a bilateral symmetry model, meaning that there was equal probability of missing teeth from the right and left sides. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  11. Use of physical therapy to augment dilator treatment for vaginal agenesis.

    Science.gov (United States)

    McVearry, Mary E; Warner, William B

    2011-05-01

    : Dilator therapy has been used successfully for many years to treat vaginal agenesis. Our objective was to show how established physical therapy techniques can be used to augment dilator therapy. : A 36-year-old woman desiring nonsurgical management of vaginal agenesis was instructed in the use of vaginal dilators by a pelvic-floor physical therapist. Manual stretching of the vaginal tissues was performed during office visits after application of heat and therapeutic ultrasound to the perineum. In addition, the patient's husband was taught how to perform the vaginal stretching at home in conjunction with dilator therapy. : The patient was able to attempt intercourse after 6 weeks of treatment and achieved full penetration by 9 weeks. At the completion of treatment, she scored 31.9 on the Female Sexual Function Index. The patient and her husband were very satisfied with the treatment approach, especially the encouragement and guidance received in physical therapy. : By using established physical therapy techniques in conjunction with dilator therapy, a faster time to intercourse may be achieved with high patient and spouse satisfaction. We recommend the involvement of a physical therapist specializing in the pelvic floor as an adjunct to standard dilator therapy in the treatment of vaginal agenesis.

  12. Relation between agenesis and shape anomaly of maxillary lateral incisors and canine impaction

    Directory of Open Access Journals (Sweden)

    Anísio Bueno de Carvalho

    2012-12-01

    Full Text Available OBJECTIVE: The purpose was to obtain information about the relation between agenesis and shape anomaly of maxillary lateral incisors and canine impaction. METHODS: Seventy-three patients with canine impaction and 73 control patients, without canine impaction, were evaluated. The mesiodistal distances of the maxillary lateral incisors adjacent to the impacted canines and the correspondent mandibular lateral incisors were measured. The adjacent lateral incisors were classified in: 1 - absent, 2 - small, 3 - peg-shaped, 4 - standard. RESULTS: The results showed that among the patients with impacted canines, there were 21 anomalous teeth (small and peg-shaped and among the control patients there were only three small and peg-shaped teeth, with a statistically significant difference (p = 0.001. No patients were found with impacted canines and absent lateral incisors. CONCLUSION: It was concluded that in patients with anomalous lateral incisors (small and peg-shaped there is a probability to present impacted canines and this must be considered.OBJETIVO: o objetivo foi obter informação sobre a relação existente entre a agenesia e/ou anomalia de forma de incisivos laterais superiores e impacção de caninos. MÉTODOS: foram avaliados 73 pacientes com impacção de caninos e 73 pacientes controle, sem impacção de caninos. Foram medidas as distâncias mesiodistais dos incisivos laterais superiores adjacentes aos caninos impactados e os incisivos laterais correspondentes inferiores. Os incisivos laterais adjacentes foram classificados em: 1 - ausentes; 2 - pequenos; 3 - conoides; 4 - normais. RESULTADOS: os resultados mostraram que no grupo de pacientes com caninos impactados foram encontrados 22 dentes anômalos (pequenos e conoides, e no grupo controle apenas três dentes pequenos e conoides, sendo uma diferença estatisticamente significativa (p=0,001. Não foram encontrados pacientes com canino impactado e incisivo lateral ausente. CONCLUS

  13. Agenesis of the corpus callosum and gray matter heterotopia in three patients with constitutional mismatch repair deficiency syndrome.

    Science.gov (United States)

    Baas, Annette F; Gabbett, Michael; Rimac, Milan; Kansikas, Minttu; Raphael, Martine; Nievelstein, Rutger Aj; Nicholls, Wayne; Offerhaus, Johan; Bodmer, Danielle; Wernstedt, Annekatrin; Krabichler, Birgit; Strasser, Ulrich; Nyström, Minna; Zschocke, Johannes; Robertson, Stephen P; van Haelst, Mieke M; Wimmer, Katharina

    2013-01-01

    Constitutional mismatch repair deficiency (CMMR-D) syndrome is a rare inherited childhood cancer predisposition caused by biallelic germline mutations in one of the four mismatch repair (MMR)-genes, MLH1, MSH2, MSH6 or PMS2. Owing to a wide tumor spectrum, the lack of specific clinical features and the overlap with other cancer predisposing syndromes, diagnosis of CMMR-D is often delayed in pediatric cancer patients. Here, we report of three new CMMR-D patients all of whom developed more than one malignancy. The common finding in these three patients is agenesis of the corpus callosum (ACC). Gray matter heterotopia is present in two patients. One of the 57 previously reported CMMR-D patients with brain tumors (therefore all likely had cerebral imaging) also had ACC. With the present report the prevalence of cerebral malformations is at least 4/60 (6.6%). This number is well above the population birth prevalence of 0.09-0.36 live births with these cerebral malformations, suggesting that ACC and heterotopia are features of CMMR-D. Therefore, the presence of cerebral malformations in pediatric cancer patients should alert to the possible diagnosis of CMMR-D. ACC and gray matter heterotopia are the first congenital malformations described to occur at higher frequency in CMMR-D patients than in the general population. Further systematic evaluations of CMMR-D patients are needed to identify possible other malformations associated with this syndrome.

  14. Obstructed hemivagina with pyocolpos: An unusual presentation after delivery

    Directory of Open Access Journals (Sweden)

    A.S. El-Agwany

    2016-06-01

    Case report: We describe a case of mullerian anomaly that was presented 9 years after menarche. Patient presented after delivery with offensive vaginal discharge and pelvic pressure 7 month after delivery without fever. She was diagnosed with bicornuate uterus, septate cervix along with obstructed hemivagina with pus collection and ipsilateral renal agenesis. She was successfully managed by transvaginal septum resection and drainage of pus.

  15. Functional aspects of treatment with implant-supported single crowns: a quality control study in subjects with tooth agenesis

    DEFF Research Database (Denmark)

    Goshima, Kenichi; Lexner, Michala O; Thomsen, Carsten Eckhart

    2010-01-01

    -Related Quality of Life (OHRQoL) in subjects with tooth agenesis. MATERIAL AND METHODS: In nine females and nine males (32 + or - 10 years) with agenesis treated with one to four ISSC (68% in the premolar region), the treatment effect and masticatory function were assessed. The evaluation was performed first...... after implant placement shortly before crown cementation, and again 1 month after cementation. It consisted of questionnaires [including Oral Health Impact Profile (OHIP-49)] and functional examination with plastic strips, the Dental Prescale Film and the Occluzer system, Xylitol color-changeable gum...... with ISSCs in subjects with tooth agenesis significantly increased masticatory function subjectively and clinically as well as OHRQoL. However, as the functional parameters before replacement of the teeth corresponded to values in subjects with complete dentitions, the functional importance of the increase...

  16. Prosthetic replacement vs space closure for maxillary lateral incisor agenesis: A systematic review.

    Science.gov (United States)

    Silveira, Giordani Santos; de Almeida, Natália Valli; Pereira, Daniele Masterson Tavares; Mattos, Cláudia Trindade; Mucha, José Nelson

    2016-08-01

    Defining the best treatment for maxillary lateral incisor agenesis is a challenge. Our aim in this study was to determine, with the evidence available in the literature, the best treatment for maxillary lateral incisor agenesis in the permanent dentition, evaluating the esthetic, occlusal (functional), and periodontal results between prosthetic replacement and orthodontic space closure. Electronic databases (CENTRAL, PubMed, Web of Science, Scopus, and LILACS) were searched in September 2014 and updated in January 2015, with no restriction on language or initial date. A manual search of the reference lists of the potential studies was performed. Risk of bias was assessed by the Newcastle Ottawa Scale. The search identified 2174 articles, of which 1196 were excluded because they were duplicates. Titles and abstracts of 978 articles were accessed, and 957 were excluded. In total, 21 articles were read in full, and 9 case-control studies were included after applying the inclusion and exclusion criteria. Data were extracted from the articles selected, and a table was compiled for comparison and analysis of the results. There were no randomization and blinding, and the risk of bias evaluation found gaps in compatibility and outcome domains in almost all selected studies. Tooth-supported dental prostheses of maxillary lateral incisor agenesis had worse scores in the periodontal indexes than did orthodontic space closure. Space closure is evaluated better esthetically than prosthetic replacements, and the presence or absence of a Class I relationship of the canines showed no relationship with occlusal function or with signs and symptoms of temporomandibular disorders. Copyright © 2016 American Association of Orthodontists. Published by Elsevier Inc. All rights reserved.

  17. Agenesia de veia cava inferior associada à trombose venosa profunda Agenesis of inferior vena cava associated with deep venous thrombosis

    Directory of Open Access Journals (Sweden)

    Clovis Luis Konopka

    2010-09-01

    Full Text Available A agenesia da veia cava inferior é uma anomalia congênita rara, que foi recentemente identificada como um importante fator de risco para o desenvolvimento e a recorrência de trombose venosa profunda de membros inferiores em jovens. O objetivo deste trabalho foi relatar o caso de uma paciente que apresentou trombose venosa profunda dois meses após a realização de cirurgia de varizes. A angiotomografia computadorizada demonstrou a presença de anomalia venosa complexa com ausência da veia cava inferior.The agenesis of the inferior vena cava is a rare congenital anomaly, which was recently identified as an important risk factor for the development and recurrence of deep venous thrombosis especially in young people. The goal of this work was to report the case of a patient who presented deep venous thrombosis approximately two months after varicose vein surgery. The computerized angiotomography demonstrated the presence of a complex venous anomaly with absence of the inferior vena cava.

  18. Fetal bilateral renal agenesis, phocomelia, and single umbilical artery associated with cocaine abuse in early pregnancy.

    Science.gov (United States)

    Kashiwagi, Maki; Chaoui, Rabih; Stallmach, Thomas; Hürlimann, Sandra; Lauper, Urs; Hebisch, Gundula

    2003-11-01

    Maternal cocaine abuse in pregnancy is associated with complications such as intrauterine growth retardation, abruptio placentae, and preterm delivery. We report what is, to our knowledge, the first published observation of fetal bilateral renal agenesis associated with a vascular disruption syndrome comprising upper limb reduction defect and a single umbilical artery following maternal cocaine abuse in early pregnancy. This constellation in a fetus aborted at 18 weeks extends the spectrum of complications possibly associated with cocaine abuse in pregnancy. Copyright 2003 Wiley-Liss, Inc.

  19. Cryptophthalmos and Bilateral Renal Agenesis with Cleft Lip and Palate: Fraser Syndrome: Case Report

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    Emre Pabuçcu

    2012-12-01

    Full Text Available Fraser syndrome is a rare autosomal recessive disorder consisting of multiple anomalies including variable expression of cryptophthalmos, syndactyly, abnormal genitalia, malformations of the nose, ear and larynx, renal agenesis, oro-facial clefts, skeletal defects, umbilical hernia and mental retardation. Antenatally detected multiple congenital fetal anomalies during 22nd week of gestation is reported in this paper. Fraser Syndrome was diagnosed according to major and minor criteria. Early antenatal detection is mandatory and clinician should be awere of the high recurrence rates of this syndrome among siblings threatening subsequent pregnancies and should inform affected families.

  20. Bilateral renal agenesis, a severe anomaly in a premature infant with VACTERL association: A case report

    Directory of Open Access Journals (Sweden)

    Erol Basuguy

    2017-11-01

    Full Text Available We report on a preterm male (birth weight 1,100 g with bilateral renal agenesis, a lethal malformation. Additionally, the child suffered from an atrial septal defect, ventricular septal defect, right aortic arch anomaly, a high type of anal atresia, vertebral anomalies, limbs defects (VACTERL association. The infant during first day of life was treated with an emergency sigmoid ostomy and peritoneal dialysis because of increasing abdominal dilatation and high urea and creatinine levels in blood. Important congenital anomalies associated with VACTERL association and prematurity are very serious causes of mortality in the early period

  1. A case of obstructed hemivagina with ipsilateral renal agenesis

    African Journals Online (AJOL)

    A.S. El-agwany

    The patient is usually young and presents with dysmenorrhea. The diagnosis is frequently delayed. Early diagnosis and excision of the vaginal septum will relieve the patient of her symptoms and prevents subsequent development of endometriosis and infertility. Case report. 22-year-old female presented to our clinic with ...

  2. Complex malformations of the urogenital tract in a female dog: Gartner duct cyst, ipsilateral renal agenesis, and ipsilateral hydrometra.

    Science.gov (United States)

    Fujita, Atsushi; Tsuboi, Masaya; Uchida, Kazuyuki; Nishimura, Ryohei

    2016-05-01

    A 10-month-old female toy poodle was referred to the University of Tokyo Veterinary Medical Center with a urogenital anomaly found during sterilization. An exploratory laparotomy revealed a cyst adhering to the cervix and a unilateral renal agenesis. Histopathology and immunohistochemical analysis of the cyst was consistent with remnants of the Wolffian duct or a Gartner duct cyst. This is a rare case of a canine Gartner duct cyst with renal agenesis and uterine anomaly. We discuss the similarity of this case to that of humans and introduce a classification in the literature for these complex urogenital malformations for further clinical research into the precise diagnosis and appropriate surgical planning.

  3. Orthodontic management of bilateral maxillary canine-first premolar transposition and bilateral agenesis of maxillary lateral incisors: a case report.

    Science.gov (United States)

    Di Palma, Elena; Di Giuseppe, Biagio; Tepedino, Michele; Chimenti, Claudio

    2015-01-01

    Maxillary canine-first premolar transposition (Mx.C.P1) is an uncommon dental positional anomaly that may create many orthodontic problems from both esthetic and functional points of view. In this report we show the orthodontic management of a case of Mx.C.P1 associated with bilateral maxillary lateral incisor agenesis and unilateral mandibular second premolar agenesis The patient was treated with a multibracket appliance and the extraction of the lower premolar. treatment was completed without the need for any prosthetic replacement.

  4. Laparoscopic Colectomy for a Patient with Congenital Renal Agenesis

    African Journals Online (AJOL)

    We present a very rare case of laparoscopic colectomy for a patient with ascending colon cancer and an agenetic right kidney. A 57‑year‑old man visited our institute for further evaluation for a positive fecal occult blood test. Approximately, 20 years earlier, the right kidney of the patient was found to be congenitally absent.

  5. Congenital tricuspid valve disease and testicular agenesis: a case ...

    African Journals Online (AJOL)

    This is a report of a case of congenital tricuspid valve disease presenting with heart failure and pulmonary hypertension. Cardinal clinical features include breathlessness, easy fatigability since childhood, stunted growth, cyanosis, finger clubbing, a pansystolic murmur loudest at the left sternal edge in the fourth intercostal ...

  6. Nonsyndromic tooth agenesis patterns and associated developmental dental anomalies: a literature review with radiographic illustrations.

    Science.gov (United States)

    Agarwal, P; Vinuth, D P; Dube, G; Dube, P

    2013-01-01

    Tooth agenesis is one of the most intriguing phenomena, because it is frequently associated with other oral anomalies, structural variations and malformations of other teeth, late eruption, transposition and crowding. The diagnosis can be quite challenging as radiographic examination is critical for the diagnosis but not always possible and the late developing teeth may be sometimes scored developmentally missing. Accurate diagnosis therefore requires radiographic, clinical, and dental cast examinations. It is an important clinical and public health problem. Patients with missing permanent teeth may suffer from a reduced chewing ability, inarticulate pronunciation, and an unfavorable aesthetic appearance. Clinically, early diagnosis of a dental anomaly can alert the clinician to the possible development of other associated dental anomalies in the same patient or family, and avoid the possible sequelae. Understanding of tooth agenesis patterns and their impact on diagnosis, prevention, and eventually therapeutics are becoming integral parts of comprehensive dental care. Dental examination with radiographic screening of hypodontia in early childhood should be emphasized as part of public oral health policy to allow early diagnosis and timely intervention.

  7. Prevalence and distribution of dental anomalies: a comparison between maxillary and mandibular tooth agenesis.

    Science.gov (United States)

    Al-Abdallah, Mariam; AlHadidi, Abeer; Hammad, Mohammad; Al-Ahmad, Hazem; Saleh, Raja'

    2015-11-01

    The aim of this study was to compare the pattern and prevalence of associated dental anomalies between maxillary and mandibular tooth agenesis (hypodontia). A sample of 3315 dental patients, aged 8.6 to 25.4 years, was surveyed for tooth agenesis (excluding third molars): 106 subjects were diagnosed with maxillary hypodontia (group 1) and 70 with mandibular hypodontia (group 2). Both groups were examined for the following dental anomalies: retained deciduous molars, infraocclusion of deciduous molars, impaction, microdontia of maxillary lateral incisors, supernumerary teeth, transposition, transmigration, and ectopic eruption of the permanent molars. For statistical testing, the chi-square test (P anomalies among the groups. We found that 77.5% of the patients in the mandibular hypodontia group had at least 1 dental anomaly compared with 49.5% in the maxillary hypodontia group (P dental anomaly with a significantly increased prevalence in the maxillary hypodontia group compared with the mandibular hypodontia group was microdontia of the maxillary lateral incisors (groups 1, 46.7%; group 2, 12.9%; P dental development in both jaws. Copyright © 2015 American Association of Orthodontists. Published by Elsevier Inc. All rights reserved.

  8. Uterus Dysplasia Associated with Cervico-Vaginal Agenesis

    Directory of Open Access Journals (Sweden)

    Ali Mahdavi

    2017-10-01

    Full Text Available Müllerian ducts can form upper parts of normal female reproductive system and any failure in ductal fusion may result in to müllerian duct anomalies (MDA. We present a case of MDA and a uterus dysplasia with no evidence of cervical or upper vaginal tissue. This case showes the role of magnetic resonace imaging (MRI on MDA diagnosis and urges the need for a unified reliable and practical classification more compatible with clinical practice.

  9. A case of Klippel-Feil syndrome with renal agenesis

    Directory of Open Access Journals (Sweden)

    Mehmet Karakoç

    2010-05-01

    Full Text Available Klippel-Feil syndrome (KFS is is a rare syndrome that characterized by fusion of at least two congenital vertebrae in the cervical region. The most common characteristics of KFS are short neck, low nuchal hair line and restricted joint mobility of the cervical region.Addititonal features are scoliosis, sipina bifida, cervical rib, Sprengel deformity and facial asymmetry, and renal and cardiac anomalies. In this report a 10 years old girl was admitted with complaints of short stature and abnormal neck curvature. Physical examination of the patient revealed short neck, low nuchal hair line, and Sprengel’s deformity, height hard palate, facial asymmetry and torticollis. Cervical paravertebral muscle spasm was found on the left as (+ / right (+ +. Muscle tone, muscle strength, reflexes and sensory examination were found to be normal. Radiological examination of the C4-5 and C6-7 cervical region revealed the loss of inteverebral disc spaces and block vertebra. Also there was hemivertebra anomaly and aplasia of odontoid process. Abdominal and pelvic ultrasound examination revelaed absence of right kidney. Echocardiographical examination gave no abnormality. The case was presented in order to draw attention to KFS with renal agenesia in the light of current literature.

  10. [Is there either agenesis or regression of the Mullerian duct in female bird embryos under the influence of male hormone?].

    Science.gov (United States)

    Lutz-Ostertag, Y; Lutz, H

    1976-01-01

    The natural occurence of "Free-Martinism" in Birds and the chorio-allantoïc grafting experiments of testis fragments on female chick host-embryos allow to the authors to define the manner provoking the entire or partial disappearance of the müllerian ducts and to state exactly if the phenomenon is a agenesis or a regression.

  11. Radiographic assessment of agenesis, impaction, and pararadicular radiolucencies in relation with third molar in Nashik City of Maharashtra

    Directory of Open Access Journals (Sweden)

    Bhushan Sukdeo Ahire

    2016-01-01

    Full Text Available Introduction: The third molar develops entirely after birth and also it is the last tooth to erupt in all ethnic groups despite racial variations in the eruption sequence. Tooth development with effects on tooth size, shape, position, and total absence is affected due to environmental factors, systemic diseases, genetic polymorphisms, and teratogens. Aims and Objectives: The aim of this study was to assess the prevalence of agenesis, impaction, and pararadicular radiolucencies in relation with third molars. Materials and Methods: One hundred digital orthopantomograph (OPG scans of patients of age ranging from 18 to 25 years were selected randomly from the digital OPG database of the Department of Oral Medicine and Radiology, and then assessment and analysis were carried out. Results: Overall agenesis rate was about 14.5%. Bilateral agenesis of maxillary and mandibular third molars was about 14% and 13%, respectively. Overall impaction rate was about 21.92%. Mesioangular impaction rate was about 57.33% whereas distoangular impaction rate was about 26.66%. Prevalence of pararadicular radiolucencies was only about 0.8% with mandibular predilection. Conclusion: As the agenesis of the third molars is increasing, they can be considered as vestigial. Prevalence of impaction of the third molars is increasing. Pararadicular radiolucencies are mostly seen with mandibular third molar.

  12. Analysis of the golden proportion and width/height ratios of maxillary anterior dentition in patients with lateral incisor agenesis.

    Science.gov (United States)

    Pini, Núbia Pavesi; de-Marchi, Luciana Manzotti; Gribel, Bruno Frazão; Ubaldini, Adriana Lemos Mori; Pascotto, Renata Corrêa

    2012-12-01

      The purpose of this study was to assess the presence of the golden proportion (GP) in the facial view tooth-to-tooth width proportion of the six maxillary anterior teeth and to evaluate the width/height (W/H) ratios of the incisors of patients with maxillary lateral incisor (LI) agenesis treated either with implants or orthodontically (by moving canines into the position of the laterals, recontouring them, and placing composite restorations over the repositioned teeth).   Forty-eight patients with LI agenesis were divided into four experimental groups: unilateral recontouring group (N = 10), bilateral recontouring group (BRG, N = 18), unilateral implant group (UIG, N = 10), bilateral implant group (N = 10), and a control group (CG, N = 25) of patients without agenesis. GP ratios were determined on patients' dental casts placed over Levin's grids, whereas W/H ratios were measured directly on the casts and a millimeter ruler to determine these distances. Statistical analysis was performed with Shapiro-Wilk, Kruskal-Wallis, Mann-Whitney, Friedman, and Wilcoxon tests (p mean W/H ratios of the laterals ranged between 0.75 and 0.90.   Although the GP may be a useful diagnostic guide, it was not observed in the majority of esthetic outcomes of patients treated with maxillary LI agenesis in this study. The assessment of the golden proportion and width/height ratio of upper anterior teeth in patients with upper lateral incisor agenesis treated with either implants or tooth re-contouring may assist dentists and patients in deciding the best treatment option based on the peculiarities of each case. © 2012 Wiley Periodicals, Inc.

  13. Prevalência de agenesia do osso sesamóide da articulação metacarpofalangeana, em brasileiros adultos Agenesis prevalence of the adductor sesamoid of the thumb in Brazilians adults

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    Renata Rodrigues de Almeida-Pedrin

    2008-08-01

    Full Text Available OBJETIVO: diante da importância do sesamóide no emprego de métodos simplificados para a determinação da maturidade esquelética, julgamos necessário um estudo, em brasileiros adultos, para verificar a porcentagem de presença ou ausência deste ossículo. METODOLOGIA: a amostra constitui-se de 500 indivíduos brasileiros, com idade média de 22,5 (18-33 anos, de ambos os gêneros. A presença ou ausência do osso sesamóide foi verificada mediante a interpretação de radiografias periapicais, tomadas da articulação metacarpofalangena dos dois polegares de cada indivíduo da pesquisa. RESULTADOS E CONCLUSÃO: os resultados demonstraram que o osso sesamóide da articulação metacarpofalangeana esteve presente em todos os indivíduos (100% da amostra, concluindo, desta forma, que não houve agenesia deste osso nesta população avaliada.AIM: the purpose of this study was to verify the percentage of agenesis of sesamoid bone in a Brazilian adult population. METHODS: the sample consisted of 329 females and 171 males, with mean age of 22.5 (range from 18 to 33 years. The presence or agenesis of sesamoid bone was evaluated through periapical radiographs taken from the metacarpophalangeal joint of the thumb (left and right of each subject. RESULTS AND CONCLUSION: The results evidenced that the sesamoid bone was present in 100% of the sample, showing that was not found agenesis in these population.

  14. Mullerian agenesis associated with in-utero thalidomide exposure: A case report

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    Sarah Dotters-Katz

    2013-09-01

    Full Text Available Thalidomide is a well-known teratogen, which is experiencing resurgence as new uses are identified. Exposure is classically associated with limb deformities, such as: dysmelia, phocomelia, preaxial hypoplasia and polydactyly, in addition to visceral anomalies that have been documented as well. We report a case of a 38 year-old nulligravid female, who was previously evaluated for primary amenorrhea, and given the presumptive false diagnosis of an imperforate hymen. On magnetic resonance imaging (MRI exam, she was noted to have uterovaginal agenesis. The implications of thalidomide on women’s health extend beyond external birth defects. Although, most commonly associated with limb deformities, there may also be gynecologic implications of in utero exposure. As this medication is increasingly used for various medical conditions, obstetricians/gynecologists need to remain aware of this potential mullerian teratogenic effect.

  15. EARS2 mutations cause fatal neonatal lactic acidosis, recurrent hypoglycemia and agenesis of corpus callosum.

    Science.gov (United States)

    Danhauser, Katharina; Haack, Tobias B; Alhaddad, Bader; Melcher, Marlen; Seibt, Annette; Strom, Tim M; Meitinger, Thomas; Klee, Dirk; Mayatepek, Ertan; Prokisch, Holger; Distelmaier, Felix

    2016-06-01

    Mitochondrial aminoacyl tRNA synthetases are essential for organelle protein synthesis. Genetic defects affecting the function of these enzymes may cause pediatric mitochondrial disease. Here, we report on a child with fatal neonatal lactic acidosis and recurrent hypoglycemia caused by mutations in EARS2, encoding mitochondrial glutamyl-tRNA synthetase 2. Brain ultrasound revealed agenesis of corpus callosum. Studies on patient-derived skin fibroblasts showed severely decreased EARS2 protein levels, elevated reactive oxygen species (ROS) production, and altered mitochondrial morphology. Our report further illustrates the clinical spectrum of the severe neonatal-onset form of EARS2 mutations. Moreover, in this case the live-cell parameters appeared to be more sensitive to mitochondrial dysfunction compared to standard diagnostics, which indicates the potential relevance of fibroblast studies in children with mitochondrial diseases.

  16. Sirenomelia: A Rare Presentation

    Science.gov (United States)

    Srinivas, S; Kumar, Shiva; Reddy, Surweshwar; Prasad, Hari; Irfan, G M

    2012-01-01

    We are presenting two cases of Sirenomelia (Mermaid Syndrome), which is an extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, imperforate anus, and renal agenesis or dysgenesis. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. One of our cases survived for 12 days after birth. This new born had an unusually high anorectal anomaly in which the colon was ending at the level of mid transverse colon, fused lower limbs and genital anomalies. Ultrasound of the abdomen revealed horseshoe kidney. Colostomy was performed on day 2 of life. The second case encountered was a stillborn baby on whom an autopsy was performed. PMID:26023366

  17. Sirenomelia: a rare presentation.

    Science.gov (United States)

    Reddy, K Ramesh; Srinivas, S; Kumar, Shiva; Reddy, Surweshwar; Prasad, Hari; Irfan, G M

    2012-01-01

    We are presenting two cases of Sirenomelia (Mermaid Syndrome), which is an extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, imperforate anus, and renal agenesis or dysgenesis. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. One of our cases survived for 12 days after birth. This new born had an unusually high anorectal anomaly in which the colon was ending at the level of mid transverse colon, fused lower limbs and genital anomalies. Ultrasound of the abdomen revealed horseshoe kidney. Colostomy was performed on day 2 of life. The second case encountered was a stillborn baby on whom an autopsy was performed.

  18. Herlyn–Werner–Wunderlich syndrome presenting with infertility: Role of MRI in diagnosis

    International Nuclear Information System (INIS)

    Ahmad, Zohra; Goyal, Ankur; Das, Chandan J; Deka, Dipika; Sharma, Raju

    2013-01-01

    Herlyn-Werner-Wunderlich syndrome (HWWS), characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, is an uncommon combined Mullerian and mesonephric duct anomaly, and its presentation in adulthood is even rarer. We report here a 22-year-old female presenting with primary infertility where magnetic resonance imaging (MRI) suggested the diagnosis of HWWS with endometriosis. In a patient of infertility with endometriosis and unilateral renal agenesis, diagnosis of HWWS should be suspected and MRI is the investigation of choice for such anomalies

  19. Associação de agenesia sacrococcígea e atresia anal em gato sem raça definida Sacrococcygeal agenesis association and anal atresia in mixed breed cats

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    Felipe Purcell de Araújo

    2009-09-01

    Full Text Available O presente trabalho teve como objetivo descrever o caso de um felino, que desde o nascimento apresentou atresia anal, ausência de cauda e malformação dos membros pélvicos. Ao exame radiográfico, pôde-se observar presença de agenesia da sétima vértebra lombar, sacro e vértebras coccígeas, espinha bífida, meningocele, hiperflexão dos joelhos e desvio valgo dos tarsos, diagnosticando-se agenesia sacrococcígea associada à atresia anal.This paper has the objective to report a case of a cat that since birth had anal atresia, absence of tail and malformation of the pelvic member. The radiographic examination revealed agenesis of the seventh lumbar vertebra, sacral and coccygeal vertebrae, spina bifida, meningocele, hyperflexion of the knees and tarsal valgus deviation, diagnosing sacrococcygeal agenesis associated with anal atresia.

  20. Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis

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    Go Miyano

    2015-01-01

    Full Text Available We describe herein a case of unilateral pulmonary agenesis (PA with oesophageal atresia (EA/tracheoesophageal fistula (TEF that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

  1. A case of split notochord syndrome: a child with a neuroenteric fistula presenting with meningitis

    NARCIS (Netherlands)

    G.H. van Ramshorst (Gabrielle); M.H. Lequin (Maarten); G.M.S. Mancini (Grazia); C. van de Ven (C.)

    2006-01-01

    textabstractThe authors describe a case of split notochord syndrome with a neuroenteric fistula in a newborn presenting with meningitis. Associated anomalies included agenesis of the corpus callosum, short colon, malrotation, epispadias, and an abnormally high bifurcation of the abdominal aorta and

  2. A comparison of skeletal maturation in patients with tooth agenesis and unaffected controls assessed by the cervical vertebral maturation (CVM) index.

    Science.gov (United States)

    Casey, Christine; Gill, Daljit S; Jones, Steven P

    2013-12-01

    The aims of this study were to (1) investigate if there is a difference in skeletal maturation between tooth agenesis and control patients and (2) whether skeletal maturation is affected by the severity of tooth agenesis. The cervical vertebral maturation (CVM) index can be used to assess skeletal maturation. A retrospective cross-sectional study. Eastman Dental Hospital, London, UK. A total of 360 cephalograms of patients aged 9-17 years (164 males and 196 females) allocated to four subgroups (mild, moderate and severe tooth agenesis patients, and controls) were assessed retrospectively. There were 90 patients in each of the four subgroups. The skeletal maturation of each subject was assessed both quantitatively and qualitatively using the CVM index. All patients in the study were either currently receiving treatment or had been discharged from the hospital. There was no statistically significant relationship between skeletal maturation and the presence of tooth agenesis. Furthermore, there was no statistically significant relationship between the skeletal maturity of patients and different severities of tooth agenesis. The data obtained from this group of patients and using this measurement tool alone does not supply sufficient reason to reject the null hypothesis. However, it suggests that it is possible that no difference exists between the groups.

  3. Má oclusão Classe I de Angle com agenesia de incisivos laterais Angle Class I malocclusion and agenesis of lateral incisors

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    Fernanda Catharino Menezes Franco

    2011-08-01

    Full Text Available O planejamento ortodôntico de pacientes com agenesia de incisivos laterais envolve considerações estéticas e funcionais de grande relevância para um resultado clínico satisfatório. Tanto o fechamento dos espaços correspondentes aos dentes ausentes quanto sua abertura ou manutenção oferecem vantagens e desvantagens que devem ser avaliadas de acordo com as características individuais do paciente. Fatores importantes que influenciam o planejamento são o padrão esquelético, o tipo de má oclusão e a coloração e forma dos caninos, dentre outros. Neste artigo, teve-se como objetivo relatar o tratamento de uma paciente com má oclusão Classe I de Angle, agenesia de incisivos laterais, sobressaliência e sobremordida diminuídas, tendência à mordida aberta e mordida cruzada.A abordagem clínica foi realizada com expansão maxilar, seguida de fechamento dos espaços, com auxílio de ancoragem esquelética extrabucal. Esse caso foi apresentado à diretoria do Board Brasileiro de Ortodontia e Ortopedia Facial (BBO, como parte dos requisitos para a obtenção do título de Diplomado pelo BBO.Orthodontic planning for patients with agenesis of lateral incisors should include extremely relevant esthetic and functional considerations so that a satisfactory clinical result is achieved. Both space closure and space opening and maintenance have advantages and disadvantages that should be evaluated according to the patient's individual characteristics. Some of the important factors that affect planning are the skeletal pattern, the type of malocclusion and the color and shape of canines. This study reports on the treatment of a patients with Class I malocclusion and agenesis of lateral incisors, overjet, decreased overbite, and a tendency to open bite and crossbite. The clinical approach included palatal expansion followed by space closure using extraoral anchorage. This case was presented to the Committee of the Brazilian Board of Orthodontics

  4. Providers' Experiences with Vaginal Dilator Training for Patients with Vaginal Agenesis.

    Science.gov (United States)

    Patel, Vrunda; Hakim, Julie; Gomez-Lobo, Veronica; Amies Oelschlager, Anne-Marie

    2018-02-01

    To examine providers' experiences with vaginal dilator training for patients with vaginal agenesis. Anonymous electronic survey. Members of the North American Society for Pediatric and Adolescent Gynecology. How providers learn about vaginal dilator training, common techniques, and methods used for patient training, assessment of patient readiness, common patient complaints, issues leading to early discontinuation. There were a total of 55 completed survey responses of which 31 respondents (56%) had been in practice for more than 10 years. Forty-nine were gynecologists (89%), 20 had completed a fellowship in pediatric and adolescent gynecology (36%), and 6 were reproductive endocrinologists (11%). Thirty-one respondents had first learned about vaginal dilator training through lectures (56%) whereas only 9 through mentorship and fellowship (16%). According to respondents, the most common issue leading to early discontinuation was lack of patient motivation and readiness (n = 42; 76%). The most common complication was pain or discomfort (n = 45; 82%). More than half of respondents determined dilator therapy was successful when patients reported comfortable sexual intercourse (n = 30; 55%) and 65% (n = 35) did not delineate any restrictions to initiation of sexual intercourse. Most respondents (87%) requested further vaginal dilator training at either a clinical meeting (n = 26; 47%) or with a training video (n = 22; 40%). Our study in an experienced cohort of pediatric gynecology providers highlights the need for further research and training on vaginal dilation education. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  5. Successful radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia in a patient with dextrocardia due to unilateral pulmonary agenesis: a case report

    Directory of Open Access Journals (Sweden)

    Aksu T

    2015-02-01

    Full Text Available Tolga Aksu, Tumer Erdem Guler, Ebru Golcuk, Ismail Erden, Kazim Serhan Ozcan Department of Cardiology, Kocaeli Derince Education and Research Hospital, Derince, Kocaeli, Turkey Abstract: Radiofrequency catheter ablation of the slow pathway is considered to be the treatment of choice for patients with atrioventricular nodal reentrant tachycardia. We report a 34-year-old female with mirror image dextrocardia due to unilateral pulmonary agenesis who underwent successful slow pathway ablation for typical atrioventricular nodal reentrant tachycardia. Using contrast injection, cardiac anatomy was identified in a short time and successfully ablated. Keywords: dextrocardia, AVNRT, ablation, pulmonary agenesis

  6. The presentation and management of complex female genital malformations.

    Science.gov (United States)

    Acién, Pedro; Acién, Maribel

    2016-01-01

    Common uterine anomalies are important owing to their impact on fertility, and complex mesonephric anomalies and certain Müllerian malformations are particularly important because they cause serious clinical symptoms and affect woman's quality of life, in addition to creating fertility problems. In these cases of complex female genital tract malformations, a correct diagnosis is essential to avoid inappropriate and/or unnecessary surgery. Therefore, acquiring and applying the appropriate embryological knowledge, management and therapy is a challenge for gynaecologists. Here, we considered complex malformations to be obstructive anomalies and/or those associated with cloacal and urogenital sinus anomalies, urinary and/or extragenital anomalies, or other clinical implications or symptoms creating a difficult differential diagnosis. A diligent and comprehensive search of PubMed and Scopus was performed for all studies published from 1 January 2011 to 15 April 2015 (then updated up to September 2015) using the following search terms: 'management' in combination with either 'female genital malformations' or 'female genital tract anomalies' or 'Müllerian anomalies'. The MeSH terms 'renal agenesis', 'hydrocolpos', 'obstructed hemivagina' 'cervicovaginal agenesis or atresia', 'vaginal agenesis or atresia', 'Herlyn-Werner-Wunderlich syndrome', 'uterine duplication' and 'cloacal anomalies' were also used to compile a list of all publications containing these terms since 2011. The basic embryological considerations for understanding female genitourinary malformations were also revealed. Based on our experience and the updated literature review, we studied the definition and classification of the complex malformations, and we analysed the clinical presentation and different therapeutic strategies for each anomaly, including the embryological and clinical classification of female genitourinary malformations. From 755 search retrieved references, 230 articles were analysed and

  7. The relationship between smile attractiveness and esthetic parameters of patients with lateral agenesis treated with tooth recontouring or implants

    Science.gov (United States)

    De-Marchi, Luciana Manzotti; Pini, Núbia Inocencya Pavesi; Pascotto, Renata Corrêa

    2012-01-01

    Background The purpose of this study was to associate smile esthetic judgment with dentofacial attributes of patients with unilateral and bilateral agenesis of maxillary lateral incisors treated with recontouring of canines or implants and patients with no agenesis (control). Material and methods Forty-six participants were divided into two groups: those treated with recontouring (N = 26) and those treated with implants (N = 20). The participants in the control group (N = 22) were selected among dentistry students at the State University of Maringá, Brazil. Photographs of posed smiles (17 cm × 10 cm) were evaluated with a 100-mm Visual Analog Scale. Smile attractiveness was judged by two groups: laypersons and dentists (N = 20 in each group). Judgment was classified into Unpleasant and Pleasant. Measurements of 11 smile attributes were done with ImageTool Version 3.0. These measurements were correlated with the type of judgment using the Pearson correlation coefficient. Results The two groups of evaluators showed no rating difference (analysis of variance, P = 0.64), thus they were placed into a single group. No significant correlation was found between esthetic judgment and six smile attributes (incisor exposure, interlabial gap, width 3 to 3, smile index, right buccal corridor, and buccal corridor ratio). The control group showed more correlations with the unpleasant judgment type than the other groups. Conclusion Some correlations between smile attributes and esthetic judgment were found, but other features of smiles not evaluated in this study may interfere in smile attractiveness. PMID:23674925

  8. Agenesis of internal carotid artery associated with isolated growth hormone deficiency: a case report and literature review.

    Science.gov (United States)

    Stagi, Stefano; Traficante, Giovanna; Lapi, Elisabetta; Pantaleo, Marilena; Becciani, Sabrina; Mortilla, Marzia; Seminara, Salvatore; de Martino, Maurizio

    2015-10-19

    Agenesis of the internal carotid artery (ICA) is a rare congenital abnormality, sporadically reported to be associated with a combined congenital hypopituitarism. Nevertheless, only a few cases have been extensively described, and none of these have been characterized by an isolated growth hormone (GH) deficiency. Here, we describe a 17-year old boy referred to our hospital for fatigue, decreased muscle strength and severe headache reported after the cessation of rhGH treatment for a GH deficiency diagnosed at the age of 2 years and 3 months. Magnetic resonance imaging (MRI) showed an adenohypophyseal hypoplasia with a lack of posterior pituitary hyperintensity, whereas MRI angiography indicated the absence of a normal flow void in the left ICA. Endocrinological tests confirmed the GH deficiency (GH peak after growth-hormone-releasing hormone (GHRH) + arginine: 2.42 ng/mL) with a very low IGF-I value (31 ng/mL) and normal function of other pituitary axes. To the best of our knowledge this is the first confirmed case of an isolated GH deficiency in a patient with ICA agenesis. The presence of an isolated pituitary deficit is unlike to be considered only as an effect of hemodynamic mechanism, suggesting a role for genetic factor(s) as a common cause of these two rare birth defects. Further studies could clarify this issue and the underlying mechanisms to better understand the etiopathogenetic characteristics of this disorder.

  9. Hippocampal agenesis in an individual who engaged in violent criminal behaviors after discontinuing carbamazepine and paroxetine treatment.

    Science.gov (United States)

    Hanada, Hiroaki; Akiyoshi, Jotaro; Kanehisa, Masayuki; Ishitobi, Yoshinobu; Tsuru, Jusen; Tanaka, Yoshihiro; Shimomura, Tsuyoshi; Kawano, Yoshihisa

    2013-01-01

    Antidepressant discontinuation syndrome (ADS) occurs after abrupt discontinuation of an antidepressant medication. A 23-year-old man with right hippocampal agenesis demonstrated sexual crime (hypersexuality) since the age of eight and had been successfully treated with carbamazepine since the age of 13. He had required increased doses of paroxetine and carbamazepine owing to the development of an unstable affect after quitting his job. He abruptly stopped taking his medication for 3 days and his criminal behaviors re-emerged. We examined changes in brain structure and activity before and after medication cessation, using MRI and functional MRI (fMRI). The image of a girl in a swimsuit increased activity in the thalamus only after medication discontinuation. The alteration in thalamic activity might induce hypersexuality. We conclude that a primary hypersexuality had been suppressed with carbamazepine and paroxetine treatment, and the discontinuation of the medication caused the hypersexuality. © 2012 American Academy of Forensic Sciences.

  10. A Neuropsychological Profile for Agenesis of the Corpus Callosum? Cognitive, Academic, Executive, Social, and Behavioral Functioning in School-Age Children.

    Science.gov (United States)

    Siffredi, Vanessa; Anderson, Vicki; McIlroy, Alissandra; Wood, Amanda G; Leventer, Richard J; Spencer-Smith, Megan M

    2018-05-01

    Agenesis of the corpus callosum (AgCC), characterized by developmental absence of the corpus callosum, is one of the most common congenital brain malformations. To date, there are limited data on the neuropsychological consequences of AgCC and factors that modulate different outcomes, especially in children. This study aimed to describe general intellectual, academic, executive, social and behavioral functioning in a cohort of school-aged children presenting for clinical services to a hospital and diagnosed with AgCC. The influences of age, social risk and neurological factors were examined. Twenty-eight school-aged children (8 to 17 years) diagnosed with AgCC completed tests of general intelligence (IQ) and academic functioning. Executive, social and behavioral functioning in daily life, and social risk, were estimated from parent and teacher rated questionnaires. MRI findings reviewed by a pediatric neurologist confirmed diagnosis and identified brain characteristics. Clinical details including the presence of epilepsy and diagnosed genetic condition were obtained from medical records. In our cohort, ~50% of children experienced general intellectual, academic, executive, social and/or behavioral difficulties and ~20% were functioning at a level comparable to typically developing children. Social risk was important for understanding variability in neuropsychological outcomes. Brain anomalies and complete AgCC were associated with lower mathematics performance and poorer executive functioning. This is the first comprehensive report of general intellectual, academic, executive social and behavioral consequences of AgCC in school-aged children. The findings have important clinical implications, suggesting that support to families and targeted intervention could promote positive neuropsychological functioning in children with AgCC who come to clinical attention. (JINS, 2018, 24, 445-455).

  11. Rathke′s cyst with ectopic neurohypophysis presenting as severe short stature with delayed puberty

    OpenAIRE

    Deep Dutta; Ajitesh Roy; Sujoy Ghosh; Pradip Mukhopadhyay; Ranen Dasgupta; Satinath Mukhopadhyay; Subhankar Chowdhury

    2012-01-01

    Ectopic neurohypophysis (EN) is found in nearly half of children with growth hormone deficiency (GHD). Rathke's cyst (RC) is uncommon in children and when present, hypopituitarism is found in nearly half of them. We present a fourteen and half-year-old girl with severe short stature and delayed puberty who on evaluation was found to have GHD, secondary hypocortisolism, and hypogonadism. Imaging revealed hypoplastic anterior pituitary, stalk agenesis, EN at tuber cinereum and intrapituitary RC...

  12. A 54 Mb 11qter duplication and 0.9 Mb 1q44 deletion in a child with laryngomalacia and agenesis of corpus callosum

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    Lall Meena

    2011-09-01

    Full Text Available Abstract Background Partial Trisomy 11q syndrome (or Duplication 11q has defined clinical features and is documented as a rare syndrome by National Organization of Rare Disorders (NORD. Deletion 1q44 (or Monosomy 1q44 is a well-defined syndrome, but there is controversy about the genes lying in 1q44 region, responsible for agenesis of the corpus callosum. We report a female child with the rare Partial Trisomy 11q syndrome and Deletion 1q44 syndrome. The genomic imbalance in the proband was used for molecular characterization of the critical genes in 1q44 region for agenesis of corpus callosum. Some genes in 11q14q25 may be responsible for laryngomalacia. Results We report a female child with dysmorphic features, microcephaly, growth retardation, seizures, acyanotic heart disease, and hand and foot deformities. She had agenesis of corpus callosum, laryngomalacia, anterior ectopic anus, esophageal reflux and respiratory distress. Chromosome analysis revealed a derivative chromosome 1. Her karyotype was 46,XX,der(1t(1;11(q44;q14pat. The mother had a normal karyotype and the karyotype of the father was 46,XY,t(1;11(q44;q14. SNP array analysis showed that the proband had a 54 Mb duplication of 11q14q25 and a 0.9 Mb deletion of the submicroscopic subtelomeric 1q44 region. Fluorescence Insitu Hybridisation confirmed the duplication of 11qter and deletion of 1qter. Conclusion Laryngomalacia or obstruction of the upper airway is the outcome of increased dosage of some genes due to Partial Trisomy 11q Syndrome. In association with other phenotypic features, agenesis of corpus callosum appears to be a landmark phenotype for Deletion 1q44 syndrome, the critical genes lying proximal to SMYD3 in 1q44 region.

  13. Agenesis of the internal carotid artery with a trans-sellar anastomosis: CT and MRI findings in late-onset congenital hypopituitarism

    International Nuclear Information System (INIS)

    Mellado, J.M.; Merino, X.; Ramos, A.; Salvado, E.; Sauri, A.

    2001-01-01

    A 29-year-old woman with a history of hypothyroidism since early childhood developed hypopituitarism. CT and MRI revealed anterior pituitary hypoplasia, an ectopic posterior lobe, a Chiari I malformation and agenesis of the right internal carotid artery with a trans-sellar anastomosis. This constellation of findings constitutes a previously unreported association in congenital hypopituitarism of late onset. The usefulness of imaging modalities and the pathogenic implications are also discussed. (orig.)

  14. Analysis of width/height ratio and gingival zenith in patients with bilateral agenesis of maxillary lateral incisor

    Directory of Open Access Journals (Sweden)

    Núbia Inocencya Pavesi Pini

    2012-10-01

    Full Text Available OBJECTIVE: The purpose of this study was to evaluate the width/length ratio and the gingival zenith (GZ, by means of dental casts and digital caliper, in patients with missing maxillary lateral incisors after treatment. METHODS: The sample was composed of 52 subjects divided into 3 groups: BRG (n = 18, patients with bilateral agenesis treated with tooth re-contouring; BIG (n = 10 patients with agenesis treated with implants and CG (n = 24, control group. The data were analyzed using Shapiro-Wilk, Spearman correlation, Wilcoxon, Kruskal-Wallis, t test and ANOVA tests (p 0.05. GZ data for the right and left sides of the smile were not considered statistically different. CONCLUSION: Although no statistical difference was found in the comparison between the groups, analysis of the descriptive values showed that group BIG showed the greatest difference in values with regard to width/length ratio. Regarding gingival zenith, BRG showed the greatest difference.OBJETIVO: o propósito desse estudo foi analisar, por meio de modelos de estudo e paquímetro digital, a proporção largura/altura e o zênite gengival (ZG em pacientes com agenesia bilateral do incisivo lateral superior após o tratamento. MÉTODOS: a amostra consistiu de 52 voluntários divididos em 3 grupos: GBR (n=18, pacientes com agenesia bilateral tratados com reanatomizações dentárias; GBI (n=10, pacientes com agenesia bilateral tratados com implantes; e GC (n=24, grupo controle. Os dados foram avaliados por meio dos testes de Shapiro-Wilk, Wilcoxon, Kruskal-Wallis, teste t, ANOVA (p0,05. Os valores obtidos para os lados direito e esquerdo foram considerados iguais dentro de cada grupo. CONCLUSÃO: embora não tenham sido encontradas diferenças estatísticas na comparação entre os grupos, pela análise descritiva dos dados, o GBI foi o grupo que apresentou as medidas mais destoantes dos demais em relação à proporção largura/altura, sendo que, para o zênite gengival, a maior

  15. Imperfuração anal associada à agenesia parcial do sacro e lipoma pré-sacral: síndrome de Currarino Imperforate anus associated with partial sacral agenesis and presacral lipoma: Currarino syndrome

    Directory of Open Access Journals (Sweden)

    Paulo Ricardo G. Zen

    2010-09-01

    anus and recto-vestibular fistula diagnosed in the first day after birth. At seven months of age, she started to present episodes of recurrent urinary infections and received a diagnosis of neurogenic bladder. At the same time, partial sacral agenesis was noted. Magnetic resonance imaging and computed tomography scan of the spine identified the presence of a fistula coincident with the lombo-sacral dimple described at clinical examination, amputation of the lower portion of the spinal cord with reduced number of nervous roots of the caudus equinus and lipomatous presacral mass. The patient did not present other dysmorphia. Parental radiologic evaluation did not identify sacral abnormalities. COMMENTS: Currarino syndrome is a rare autosomal dominant genetic disease characterized by the triad composed of anal atresia, partial sacral agenesis and presacral tumor. It includes, among others, teratomas, meningoceles, enteric cysts and lipomas, as observed in our patient. Children presenting anorectal abnormalities should be evaluated regarding the presence of Currarino syndrome. The partial sacral agenesis is a major sign of this disease.

  16. Esthetic Assessment of the Effect of Gingival Exposure in the Smile of Patients with Unilateral and Bilateral Maxillary Incisor Agenesis.

    Science.gov (United States)

    Pinho, Teresa; Bellot-Arcís, Carlos; Montiel-Company, José María; Neves, Manuel

    2015-07-01

    The aim of this study was to determine the dental esthetic perception of the smile of patients with maxillary lateral incisor agenesis (MLIA); the perceptions were examined pre- and post-treatment. Esthetic determinations were made with regard to the gingival exposure in the patients' smile by orthodontists, general dentists, and laypersons. Three hundred eighty one people (80 orthodontists, 181 general dentists, 120 laypersons) rated the attractiveness of the smile in four cases before and after treatment, comprising two cases with unilateral MLIA and contralateral microdontia and two with bilateral MLIA. For each case, the buccal photograph was adjusted using a computer to apply standard lips to create high, medium, and low smiles. A numeric scale was used to measure the esthetic rating perceived by the judges. The resulting arithmetic means were compared using an ANOVA test, a linear trend, and a Student's t-test, applying a significance level of p esthetic perception of smiles in post-treatment cases. © 2014 by the American College of Prosthodontists.

  17. Aberrant internal carotid artery presenting as a retrotympanic vascular mass

    International Nuclear Information System (INIS)

    Nicolay, Simon; De Foer, Bert; Bernaerts, Anja; Van Dinther, Joost; Parizel, Paul M

    2014-01-01

    We report a case of a young woman with an aberrant right internal carotid artery (ICA) presenting as a retrotympanic reddish mass. This variant of the ICA represents the collateral pathway that is formed as a result of an embryological agenesis of the cervical segment of the ICA. The embryonic inferior tympanic artery is recruited to bypass the absent carotid segment. This hypertrophied vessel may be seen otoscopically and wrongfully considered to be a vascular middle ear tumor. Informing the otorhinolaryngologist of this important vascular variant not only obviates biopsy but also helps in careful preoperative planning of eventual middle ear procedures

  18. Both rare and de novo copy number variants are prevalent in agenesis of the corpus callosum but not in cerebellar hypoplasia or polymicrogyria.

    Directory of Open Access Journals (Sweden)

    Samin A Sajan

    Full Text Available Agenesis of the corpus callosum (ACC, cerebellar hypoplasia (CBLH, and polymicrogyria (PMG are severe congenital brain malformations with largely undiscovered causes. We conducted a large-scale chromosomal copy number variation (CNV discovery effort in 255 ACC, 220 CBLH, and 147 PMG patients, and 2,349 controls. Compared to controls, significantly more ACC, but unexpectedly not CBLH or PMG patients, had rare genic CNVs over one megabase (p = 1.48×10⁻³; odds ratio [OR] = 3.19; 95% confidence interval [CI] = 1.89-5.39. Rare genic CNVs were those that impacted at least one gene in less than 1% of the combined population of patients and controls. Compared to controls, significantly more ACC but not CBLH or PMG patients had rare CNVs impacting over 20 genes (p = 0.01; OR = 2.95; 95% CI = 1.69-5.18. Independent qPCR confirmation showed that 9.4% of ACC patients had de novo CNVs. These, in comparison to inherited CNVs, preferentially overlapped de novo CNVs previously observed in patients with autism spectrum disorders (p = 3.06×10⁻⁴; OR = 7.55; 95% CI = 2.40-23.72. Interestingly, numerous reports have shown a reduced corpus callosum area in autistic patients, and diminished social and executive function in many ACC patients. We also confirmed and refined previously known CNVs, including significantly narrowing the 8p23.1-p11.1 duplication present in 2% of our current ACC cohort. We found six novel CNVs, each in a single patient, that are likely deleterious: deletions of 1p31.3-p31.1, 1q31.2-q31.3, 5q23.1, and 15q11.2-q13.1; and duplications of 2q11.2-q13 and 11p14.3-p14.2. One ACC patient with microcephaly had a paternally inherited deletion of 16p13.11 that included NDE1. Exome sequencing identified a recessive maternally inherited nonsense mutation in the non-deleted allele of NDE1, revealing the complexity of ACC genetics. This is the first systematic study of CNVs in congenital brain malformations, and

  19. Does the serum uric acid level have any relation to arterial stiffness or blood pressure in adults with congenital renal agenesis and/or hypoplasia?

    Science.gov (United States)

    Yazici, Raziye; Guney, İbrahim; Altintepe, Lutfullah; Yazici, Mehmet

    2017-01-01

    The relationship between serum uric acid and arterial stiffness or blood pressure is not clear. The serum uric acid level and its association with cardiovascular risk is not well known in patients with reduced renal mass. We aimed to investigate the relation between serum uric acid levels and arterial stiffness and also blood pressure in patients with congenital renal agenesis and/or hypoplasia. In this single center, cross-sectional study, a total of 55 patients (39 (% 70.9) with unilateral small kidney and 16 (%29.1) with renal agenesis) were included. The median age was 35 (21-50) years. The study population was divided into tertiles of serum uric acid (according to 2.40-3.96, 3.97-5.10, and 5.11-9.80 mg/dl cut-off values of serum uric acid levels). Official and 24-h ambulatory non-invasive blood pressures of all patients were measured. The arterial stiffness was assessed by pulse wave velocity (PWV). PWV values were increased from first to third tertile (5.5 ± 0.6, 5.7 ± 0.8, 6.1 ± 0.7, respectively), but this gradual increase between tertiles did not reach significance. Linear regression analyses showed a positive correlation between serum uric acid levels and PWV (β = 0.40, p = 0.010), but no correlation was found between uric acid and daytime systolic blood pressure (β = 0.24, p = 0.345). In congenital renal agenesis/hypoplasia, the serum uric acid level was positively correlated with arterial stiffness, but there was no correlation with blood pressure.

  20. Triplets with growth failure, microcephaly, mental retardation, nail hypoplasia and corpus callosum agenesis: is it a variant of Coffin-Siris or a new syndrome?

    Science.gov (United States)

    Kirel, B; Kural, N; Yakut, A; Adapinar, B

    2000-01-01

    We report eight-year-old triplet girls whose clinical features included microcephaly, severe mental retardation, hypoplasia of distal phalanges of both fifth and second fingers and nail hypoplasia on second fingers, dysmorphic facial features, and partial corpus callosum agenesis. During infancy, a Pavlik harness was used for congenital hip dislocation, and they had difficulty in feeding. One had been operated for patent ductus arteriosus. To our knowledge, this rare combination has not been previously reported in triplets whose clinical features closely resemble those of Coffin-Siris syndrome. The other diagnostic possibilities are also reviewed.

  1. Large Sphenoethmoidal Encephalocele Associated with Agenesis of Corpus Callosum and Cleft Palate

    Directory of Open Access Journals (Sweden)

    Basir Hashemi

    2010-06-01

    Full Text Available AbstractBasal encephalocele is a rare craniofacial anomaly. In the presentpaper we report a 10-year-old boy presented with cleftpalate, congenital nystagmus, and hypertelorism. During preoperativeevaluation for cleft palate repair, a pulsatile masswas detected in the pharynx. Magnetic resonance imagingshowed sphenoethmoidal type of basal encephalocele andagenesis of corpus callosum. Neurosurgical consultation wasperformed for further evaluation and management.Iran J Med Sci 2010; 35(2: 154-156.

  2. Self-referential and social cognition in a case of autism and agenesis of the corpus callosum

    Directory of Open Access Journals (Sweden)

    Lombardo Michael V

    2012-11-01

    Full Text Available Abstract Background While models of autism spectrum conditions (ASC are emerging at the genetic level of analysis, clear models at higher levels of analysis, such as neuroanatomy, are lacking. Here we examine agenesis of the corpus callosum (AgCC as a model at the level of neuroanatomy that may be relevant for understanding self-referential and social-cognitive difficulties in ASC. Methods We examined performance on a wide array of tests in self-referential and social-cognitive domains in a patient with both AgCC and a diagnosis of ASC. Tests included a depth-of-processing memory paradigm with self-referential and social-cognitive manipulations, self-report measures of self-consciousness, alexithymia, and empathy, as well as performance measures of first-person pronoun usage and mentalizing ability. The performance of the AgCC patient was compared to a group of individuals with ASC but without AgCC and with neurotypical controls. These comparison groups come from a prior study where group differences were apparent across many measures. We used bootstrapping to assess whether the AgCC patient exhibited scores that were within or outside the 95% bias-corrected and accelerated bootstrap confidence intervals observed in both comparison groups. Results Within the depth-of-processing memory paradigm, the AgCC patient showed decreased memory sensitivity that was more extreme than both comparison groups across all conditions. The patient’s most pronounced difficulty on this task emerged in the social-cognitive domain related to information-processing about other people. The patient was similar to the ASC group in benefiting less from self-referential processing compared to the control group. Across a variety of other self-referential (i.e. alexithymia, private self-consciousness and social-cognitive measures (i.e. self-reported imaginative and perspective-taking subscales of empathy, mentalizing, the AgCC patient also showed more extreme scores than

  3. Mayer–Rokitansky–Kuster–Hauser syndrome: Syndrome of Mullerian agenesis – A report of two cases

    Directory of Open Access Journals (Sweden)

    Sushma Yalavarthi

    2017-01-01

    Full Text Available The Mayer–Rokitansky–Kuster–Hauser syndrome (MRKH syndrome, simply called Rokitansky syndrome or vaginal aplasia of the uterus, is a congenital condition that is characterized by the absence of the uterus and vagina, but ovaries are present and the external genitalia are normal. It affects at least 1 out of 4500 women. MRKH may be isolated (Type I, but it is more frequently associated with renal, vertebral, and to a lesser extent, auditory and cardiac defects (MRKH Type II or Mullerian duct aplasia, Renal dysplasia, and Cervical Somite anomalies association - mullerian duct aplasia, renal dysplasia, and cervical somite anomalies. There were very few cases of MRKH syndrome reported in the literature. Here, we report two cases of MRKH syndrome, one in a 20-year-old woman who presented with primary amenorrhea (MRKH Type I and the other in a 65-year-old woman with primary amenorrhea and associated renal malformations and a rare ovarian sertoliform variant of endometrioid tumor (MRKH Type II.

  4. Prenatal Diagnosis of Bilateral Ectrodactyly and Radial Agenesis Associated with Trisomy 10 Mosaicism

    Directory of Open Access Journals (Sweden)

    Jonathan Lévy

    2013-01-01

    Full Text Available Ectrodactyly or split hand and foot malformations (SHFMs are rare malformations of the limbs, characterized by median clefts of the hands and feet, syndactyly, and aplasia and/or hypoplasia of the phalanges. They represent a clinically and genetically heterogeneous disorder, with both sporadic and familial cases. Most of the genomic rearrangements identified to date in some forms of SHFM are autosomal dominant traits, involving various chromosome regions. Bilateral radial ray defects comprise also a large heterogenous group of disorders, including trisomy 18, Fanconi anemia, and thrombocytopenia-absent-radius syndrome, not commonly associated with ectrodactyly. The present paper describes a case of ectrodactyly associated with bilateral radial ray defects, diagnosed in the first trimester of pregnancy, in a fetus affected by trisomy 10. Only four cases of sporadic and isolated ectrodactyly, diagnosed by ultrasonography between 14 and 22 weeks’ gestation, have been reported. To our knowledge, the present case is the first report of mosaic trisomy 10 associated with SHFM and radial aplasia. Trisomy 10 is a rare lethal chromosomal abnormality, most frequently found in abortion products. Only six liveborn mosaic trisomy 10 infants, with severe malformations, dead in early infancy, have been reported. A severe clinical syndrome can be defined, comprising ear abnormalities, cleft lip/palate, malformations of eyes, heart, and kidneys, and deformity of hands and feet and most often associated with death neonatally or in early infancy.

  5. Third Molar Agenesis, Prevalence, Distribution and Association with Other Dental Anomalies

    OpenAIRE

    Herrera-Atoche, José Rubén; Colomé-Ruiz, Gabriel Eduardo; Escoffié-Ramírez, Mauricio

    2013-01-01

    La agenesia de terceros molares se ha reportado en un 24,3% en México. La agenesia dental está asociada a otras anomalías dentales debido a su origen genético. El objetivo del presente estudio fue determinar la prevalencia y distribución de agenesia de terceros molares así como establecer su asociación con otras anomalías dentales en una población ortodóntica. La muestra incluyó 670 pacientes ortodónticos de 9 a 20 años de edad. Se excluyeron pacientes con tratamiento ortodóntico previo y con...

  6. Surgical-orthodontic treatment of Class III malocclusion with agenesis of lateral incisor and unerupted canine

    Directory of Open Access Journals (Sweden)

    Bruno Boaventura Vieira

    2013-06-01

    Full Text Available INTRODUCTION: Orthodontic-surgical treatment was performed in patient with skeletal Class III malocclusion due to exceeding mandibular growth. Patient also presented upper and lower dental protrusion, overjet of -3.0 mm, overbite of -1.0 mm, congenital absence of tooth #22, teeth #13 and supernumerary impaction, tooth #12 with conoid shape and partly erupted in supraversion, prolonged retention of tooth #53, tendency to vertical growth of the face and facial asymmetry. The discrepancy on the upper arch was -2.0 mm and -5.0 mm on the lower arch. METHODS: The pre-surgical orthodontic treatment was performed with extractions of the teeth #35 and #45. On the upper arch, teeth #53, #12 and supernumerary were extracted to accomplish the traction of the impacted canine. The spaces of the lower extractions were closed with mesialization of posterior segment. After aligning and leveling the teeth, extractions spaces closure and correct positioning of teeth on the bone bases, the correct intercuspation of the dental arch, with molars and canines in Angle's Class I, coincident midline, normal overjet and overbite and ideal torques, were evaluated through study models. The patient was submitted to orthognathic surgery and then the post-surgical orthodontic treatment was finished. RESULTS: The Class III malocclusion was treated establishing occlusal and facial normal standards.

  7. Joubert syndrome with autism in two siblings: A rare presentation.

    Science.gov (United States)

    Raghavan, D Vijaya; Doshi, V Vimal; Nambi, Shanthi

    2016-01-01

    Joubert syndrome is a rare autosomal recessive disorder with partial or complete agenesis of cerebellar vermis. This syndrome is identified mainly by the presence of molar tooth sign in magnetic resonance imaging of the brain since it has a varied phenotypic presentation. Of the 200 cases reported so far in the literature, only three reports show the presence of autistic symptoms in siblings suggesting a link between the cerebellar vermis and autistic spectrum disorders. In this case report of two siblings, the female child satisfied the criterion for autistic spectrum disorder in accordance with Diagnostic and Statistical Manual of Mental Disorders Fifth Editon. The boy showed developmental delay with autistic features (not amounting to diagnostic threshold). This report is important in that it adds evidence to the literature that abnormalities of cerebellum are involved in the cognitive development and autistic symptoms.

  8. Delivery presentations

    Science.gov (United States)

    Pregnancy - delivery presentation; Labor - delivery presentation; Occiput posterior; Occiput anterior; Brow presentation ... The mother can walk, rock, and try different delivery positions during labor to help encourage the baby ...

  9. Lecture Presentations

    International Nuclear Information System (INIS)

    2007-01-01

    The Heavy-Ion Collisions in the LHC workshop held in Cracow from 18 to 18 May 2007. The main subject of the workshop was to present the newest results of research provided at CERN LHC collider. Additionally some theoretical models and methods used for presented data analysis were discussed

  10. CATCHY PRESENTATIONS

    DEFF Research Database (Denmark)

    Eriksen, Kaare; Tollestrup, Christian; Ovesen, Nis

    2011-01-01

    An important competence for designers is the ability to communicate and present ideas and proposals for customers, partners, investors and colleagues. The Pecha Kucha principle, developed by Astrid Klein and Mark Dytham, has become a widely used and easy format for the presentation of new concepts...

  11. Novel EDA or EDAR Mutations Identified in Patients with X-Linked Hypohidrotic Ectodermal Dysplasia or Non-Syndromic Tooth Agenesis

    Directory of Open Access Journals (Sweden)

    Binghui Zeng

    2017-10-01

    Full Text Available Abstract: Both X-linked hypohidrotic ectodermal dysplasia (XLHED and non-syndromic tooth agenesis (NSTA result in symptoms of congenital tooth loss. This study investigated genetic causes in two families with XLHED and four families with NSTA. We screened for mutations of WNT10A, EDA, EDAR, EDARADD, PAX9, MSX1, AXIN2, LRP6, and WNT10B through Sanger sequencing. Whole exome sequencing was performed for the proband of NSTA Family 4. Novel mutation c.1051G>T (p.Val351Phe and the known mutation c.467G>A (p.Arg156His of Ectodysplasin A (EDA were identified in families with XLHED. Novel EDA receptor (EDAR mutation c.73C>T (p.Arg25*, known EDA mutation c.491A>C (p.Glu164Ala, and known Wnt family member 10A (WNT10A mutations c.511C>T (p.Arg171Cys and c.742C>T (p.Arg248* were identified in families with NSTA. The novel EDA and EDAR mutations were predicted as being pathogenic through bioinformatics analyses and structural modeling. Two variants of WNT10A, c.374G>A (p.Arg125Lys and c.125A>G (p.Asn42Ser, were found in patients with NSTA. The two WNT10A variants were predicted to affect the splicing of message RNA, but minigene experiments showed normal splicing of mutated minigenes. This study uncovered the genetic foundations with respect to six families with XLHED or NSTA. We identified six mutations, of which two were novel mutations of EDA and EDAR. This is the first report of a nonsense EDAR mutation leading to NSTA.

  12. Workshop presentations

    International Nuclear Information System (INIS)

    Sanden, Per-Olof; Edland, Anne; Reiersen, Craig; Mullins, Peter; Ingemarsson, Karl-Fredrik; Bouchard, Andre; Watts, Germaine; Johnstone, John; Hollnagel, Erik; Ramberg, Patric; Reiman, Teemu

    2009-01-01

    An important part of the workshop was a series of invited presentations. The presentations were intended to both provide the participants with an understanding of various organisational approaches and activities as well as to stimulate the exchange of ideas during the small group discussion sessions. The presentation subjects ranged from current organisational regulations and licensee activities to new organisational research and the benefits of viewing organisations from a different perspective. There were more than a dozen invited presentations. The initial set of presentations gave the participants an overview of the background, structure, and aims of the workshop. This included a short presentation on the results from the regulatory responses to the pre-workshop survey. Representatives from four countries (Sweden, Canada, Finland, and the United Kingdom) expanded upon their survey responses with detailed presentations on both regulatory and licensee safety-related organisational activities in their countries. There were also presentations on new research concerning how to evaluate safety critical organisations and on a resilience engineering perspective to safety critical organisations. Below is the list of the presentations, the slides of which being available in Appendix 2: 1 - Workshop Welcome (Per-Olof Sanden); 2 - CSNI Working Group on Human and Organisational Factors (Craig Reiersen); 3 - Regulatory expectations on justification of suitability of licensee organisational structures, resources and competencies (Anne Edland); 4 - Justifying the suitability of licensee organisational structures, resources and competencies (Karl-Fredrik Ingemarsson); 5 - Nuclear Organisational Suitability in Canada (Andre Bouchard); 6 - Designing and Resourcing for Safety and Effectiveness (Germaine Watts); 7 - Organisational Suitability - What do you need and how do you know that you've got it? (Craig Reiersen); 8 - Suitability of Organisations - UK Regulator's View (Peter

  13. Recurrent meningitis in a case of congenital anterior sacral meningocele and agenesis of sacral and coccygeal vertebrae Meningite recorrente em um paciente com meningocele sacral anterior e agenesia sacral e coccigea

    Directory of Open Access Journals (Sweden)

    Carolina A. R. Funayama

    1995-12-01

    Full Text Available A rare case of recurrent meningitis due to congenital anterior sacral meningocele and agenesis of the sacral and coccygeal vertebrae is described. An autosomal dominant inheritance is demonstrated for lower cord malformation, and environmental factors (chromic acid or fumes are discussed.Um caso raro de meningite recorrente devido a meningocele sacral anterior e agenesia das vértebras sacras coccígeas é descrito. Herança autossômica dominante para malformação medular caudal é demonstrada e, possíveis fatores ambientais (ligados ao cromo, são discutidos.

  14. CERN presentations

    CERN Multimedia

    CERN. Geneva

    2011-01-01

    Presentation by CERN (10 minutes each) Rolf Landua - Education and Outreach Salvatore Mele - Open Access Jean-Yves Le Meur - Digital Library in Africa Francois Fluckiger - Open Source/Standards (tbc) Tim Smith - Open Data for Science Tullio Basiglia - tbc

  15. Delivery presentations

    Science.gov (United States)

    ... has entered the pelvis. If the presenting part lies above the ischial spines, the station is reported ... M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health ...

  16. Information Presentation

    Science.gov (United States)

    Holden, K.L.; Boyer, J.L.; Sandor, A.; Thompson, S.G.; McCann, R.S.; Begault, D.R.; Adelstein, B.D.; Beutter, B.R.; Stone, L.S.

    2009-01-01

    The goal of the Information Presentation Directed Research Project (DRP) is to address design questions related to the presentation of information to the crew. The major areas of work, or subtasks, within this DRP are: 1) Displays, 2) Controls, 3) Electronic Procedures and Fault Management, and 4) Human Performance Modeling. This DRP is a collaborative effort between researchers at Johnson Space Center and Ames Research Center.

  17. Voting Present

    Directory of Open Access Journals (Sweden)

    James Lo

    2013-12-01

    Full Text Available During his time as a state senator in Illinois, Barack Obama voted “Present” 129 times, a deliberate act of nonvoting that subsequently became an important campaign issue during the 2008 presidential elections. In this article, I examine the use of Present votes in the Illinois state senate. I find evidence that Present votes can largely be characterized as protest votes used as a legislative tool by the minority party. Incorporating information from Present votes into a Bayesian polytomous item-response model, I find that this information increases the efficiency of ideal point estimates by approximately 35%. There is little evidence of significant moderation by Obama when Present votes are accounted for, though my results suggest that Obama’s voting record may have moderated significantly before his subsequent election to the U.S. Senate. My results also suggest that because legislative nonvoting may occur for a variety of reasons, naive inclusion of nonvoting behavior into vote choice models may lead to biased results.

  18. Lecture Presentations

    International Nuclear Information System (INIS)

    2008-01-01

    The Symposium on Physics of Elementary Interactions in the LHC Era held in Warsaw from 21 to 22 April 2008. The main subject of the workshop was to present the progress in CERN LHC collider project. Additionally some satellite activities in field of education, knowledge and technology transfer in the frame of CERN - Poland cooperation were shown

  19. Presentation Technique

    International Nuclear Information System (INIS)

    Froejmark, M.

    1992-10-01

    The report presents a wide, easily understandable description of presentation technique and man-machine communication. General fundamentals for the man-machine interface are illustrated, and the factors that affect the interface are described. A model is presented for describing the operators work situation, based on three different levels in the operators behaviour. The operator reacts routinely in the face of simple, known problems, and reacts in accordance with predetermined plans in the face of more complex, recognizable problems. Deep fundamental knowledge is necessary for truly complex questions. Today's technical status and future development have been studied. In the future, the operator interface will be based on standard software. Functions such as zooming, integration of video pictures, and sound reproduction will become common. Video walls may be expected to come into use in situations in which several persons simultaneously need access to the same information. A summary of the fundamental rules for the design of good picture ergonomics and design requirements for control rooms are included in the report. In conclusion, the report describes a presentation technique within the Distribution Automation and Demand Side Management area and analyses the know-how requirements within Vattenfall. If different systems are integrated, such as geographical information systems and operation monitoring systems, strict demands are made on the expertise of the users for achieving a user-friendly technique which is matched to the needs of the human being. (3 figs.)

  20. Technical presentation

    CERN Document Server

    FI Department

    2008-01-01

    RADIOSPARES, the leading catalogue distributor of components (electronic, electrical, automation, etc.) and industrial supplies will be at CERN on Friday 3 October 2008 (Main Building, Room B, from 9.00 a.m. to 3.00 p.m.) to introduce its new 2008/2009 catalogue. This will be the opportunity for us to present our complete range of products in more detail: 400 000 part numbers available on our web site (Radiospares France, RS International, extended range of components from other manufacturers); our new services: quotations, search for products not included in the catalogue, SBP products (Small Batch Production: packaging in quantities adapted to customers’ requirements); partnership with our focus manufacturers; demonstration of the on-line purchasing tool implemented on our web site in conjunction with CERN. RADIOSPARES will be accompanied by representatives of FLUKE and TYCO ELECTRONICS, who will make presentations, demonstrate materials and answer any technical questio...

  1. Stenting of the right ventricular outflow tract after thrombosis of the modified Blalock-Taussig shunt in a 8 month old infant with tetralogy of Fallot and right pulmonary artery agenesis

    Directory of Open Access Journals (Sweden)

    I. A. Soynov

    2017-01-01

    Full Text Available Pulmonary artery agenesis combined with tetralogy of Fallot is the most rarely seen congenital heart disease. Children with this anomaly are an especially problematic category of patients undergoing staged surgical repair. The postoperative period quite often is complicated with shunt thrombosis while a redo open surgery is associated with a very high risk; therefore, endovascular repair is a preferred procedure. We describe a case of right ventricular outflow tract stenting in a 8 month old girl with tetralogy of Fallot and right pulmonary artery agenesis. The patient was admitted at 3 month after performing of a left-sided modified Blalock-Taussig shunt with severe signs of heart failure and desaturation caused by shunt thrombosis. Assessments performed at 2 months after stenting of the outflow tract demonstrated good oxygen saturation in arterial blood (80% and above and improvement of heart failure symptoms to NYHA II class.

  2. Technical presentation

    CERN Document Server

    FP Department

    2009-01-01

    07 April 2009 Technical presentation by Leuze Electronics: 14.00 – 15.00, Main Building, Room 61-1-017 (Room A) Photoelectric sensors, data identification and transmission systems, image processing systems. We at Leuze Electronics are "the sensor people": we have been specialising in optoelectronic sensors and safety technology for accident prevention for over 40 years. Our dedicated staff are all highly customer oriented. Customers of Leuze Electronics can always rely on one thing – on us! •\tFounded in 1963 •\t740 employees •\t115 MEUR turnover •\t20 subsidiaries •\t3 production facilities in southern Germany Product groups: •\tPhotoelectric sensors •\tIdentification and measurements •\tSafety devices

  3. A 3-year prospective study of implant-supported, single-tooth restorations of all-ceramic and metal-ceramic materials in patients with tooth agenesis.

    Science.gov (United States)

    Hosseini, Mandana; Worsaae, Nils; Schiødt, Morten; Gotfredsen, Klaus

    2013-10-01

    The purpose of this clinical study was to describe outcome variables of all-ceramic and metal-ceramic implant-supported, single-tooth restorations. A total of 59 patients (mean age: 27.9 years) with tooth agenesis and treated with 98 implant-supported single-tooth restorations were included in this study. Two patients did not attend baseline examination, but all patients were followed for 3 years. The implants supported 52 zirconia, 21 titanium and 25 gold alloy abutments, which retained 64 all-ceramic and 34 metal-ceramic crowns. At baseline and 3-year follow-up examinations, the biological outcome variables such as survival rate of implants, marginal bone level, modified Plaque Index (mPlI), modified Sulcus Bleeding Index (mBI) and biological complications were registered. The technical outcome variables included abutment and crown survival rate, marginal adaptation of crowns, cement excess and technical complications. The aesthetic outcome was assessed by using the Copenhagen Index Score, and the patient-reported outcomes were recorded using the OHIP-49 questionnaire. The statistical analyses were mainly performed by using mixed model of ANOVA for quantitative data and PROC NLMIXED for ordinal categorical data. The 3-year survival rate was 100% for implants and 97% for abutments and crowns. Significantly more marginal bone loss was registered at gold-alloy compared to zirconia abutments (P = 0.040). The mPlI and mBI were not significantly different at three abutment materials. The frequency of biological complications was higher at restorations with all-ceramic restorations than metal-ceramic crowns. Loss of retention, which was only observed at metal-ceramic crowns, was the most frequent technical complication, and the marginal adaptations of all-ceramic crowns were significantly less optimal than metal-ceramic crowns (P = 0.020). The professional-reported aesthetic outcome demonstrated significantly superior colour match of all-ceramic over metal

  4. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome

    Directory of Open Access Journals (Sweden)

    Jane Jackie David

    2016-01-01

    Full Text Available We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner′s syndrome.

  5. A Rare Case of Mayer-Rokitansky-Kuster-Hauser Syndrome Presenting with Acute Psychosis.

    Science.gov (United States)

    Nath, Kamal; Boro, Bhanita; Naskar, Subrata

    2016-04-01

    The psychiatric co-morbidities in female population with mullerian agenesis is an area with limited research. This is probably due to the fact that when those patients are diagnosed not much attention or information is given for long term psychiatric follow-up. Owing to their inability to bear children, these subjects often become socially harassed. Thus these constant stressors may lead to development of psychopathology in future. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital abnormality with absence of uterus, cervix and vagina, but normal secondary sexual characteristics and external genitalia and occurs in every 1 out of 4000-10,000 females. There is also limited literature on the probable common chromosomal aetiology for both psychosis and MRKH patients. We, present here a case of MRKH syndrome, whose initial presentation was psychosis only. In this respect, we also highlight the much neglected need of appropriate psychiatric screening and provision of psychiatric care in this population.

  6. Rathke's cyst with ectopic neurohypophysis presenting as severe short stature with delayed puberty.

    Science.gov (United States)

    Dutta, Deep; Roy, Ajitesh; Ghosh, Sujoy; Mukhopadhyay, Pradip; Dasgupta, Ranen; Mukhopadhyay, Satinath; Chowdhury, Subhankar

    2012-12-01

    Ectopic neurohypophysis (EN) is found in nearly half of children with growth hormone deficiency (GHD). Rathke's cyst (RC) is uncommon in children and when present, hypopituitarism is found in nearly half of them. We present a fourteen and half-year-old girl with severe short stature and delayed puberty who on evaluation was found to have GHD, secondary hypocortisolism, and hypogonadism. Imaging revealed hypoplastic anterior pituitary, stalk agenesis, EN at tuber cinereum and intrapituitary RC. This is perhaps the first report of simultaneous occurrence of EN and RC, which was seen in a girl with multiple pituitary hormone deficiency. A primary defect in pituitary development may explain this simultaneous occurrence of EN and RC and hence this severe anterior pituitary function deficit.

  7. Rathke′s cyst with ectopic neurohypophysis presenting as severe short stature with delayed puberty

    Directory of Open Access Journals (Sweden)

    Deep Dutta

    2012-01-01

    Full Text Available Ectopic neurohypophysis (EN is found in nearly half of children with growth hormone deficiency (GHD. Rathke′s cyst (RC is uncommon in children and when present, hypopituitarism is found in nearly half of them. We present a fourteen and half-year-old girl with severe short stature and delayed puberty who on evaluation was found to have GHD, secondary hypocortisolism, and hypogonadism. Imaging revealed hypoplastic anterior pituitary, stalk agenesis, EN at tuber cinereum and intrapituitary RC. This is perhaps the first report of simultaneous occurrence of EN and RC, which was seen in a girl with multiple pituitary hormone deficiency. A primary defect in pituitary development may explain this simultaneous occurrence of EN and RC and hence this severe anterior pituitary function deficit.

  8. Agenesis of the Corpus Callosum

    Science.gov (United States)

    ... callosum, the structure that connects the two hemispheres (left and right) of the brain. In ACC the corpus callosum is partially or completely absent. It is caused by a disruption of brain cell migration during fetal development. ACC can occur as an isolated condition or ...

  9. Joubert syndrome presenting as unilateral dysplastic kidney, hypotonia, and respiratory problem

    Directory of Open Access Journals (Sweden)

    Majid Malaki

    2012-01-01

    Full Text Available An 8-month-old girl with a history of asphyxia and respiratory distress immediately after birth was hospitalized at her fourth month of age with the diagnosis of kidney infection and it was revealed that she had a unilateral multicystic dysplastic kidney. In recent admission, she presented to emergency room with fever, hyperpnea, and apnea. In appearance, she was a hypotonic girl with broad forehead, hypertelorism, depressed nasal bridge and bitemporal regions, rapid vertical and horizontal nystagmus, and open mouth with salivation. In spite of normal physical growth, she had delayed developmental milestones. Blood gas O 2 saturation dropped after she received phenobarbital. Her urinary and blood tests were normal; however, her cranial magnetic resonance imaging (MRI revealed vermis agenesis and molar tooth sign. These physical and para-clinical findings suggested Joubert syndrome.

  10. Case report 332: A complex anomaly of the craniovertebral junction representing a regressive malformation with agenesis of the neural arch of C-2, hypomorphogenesis at C5-C6 and instability of the upper cervical spine

    International Nuclear Information System (INIS)

    Bernini, F.P.; Muras, I.

    1985-01-01

    In summary, a very complex anomaly, including agenesis of the neural arch of the axis, hypomorphogenesis of the cervical spine at the C5-C6 level with a partial ''blocked'' vertebra and disability of the upper cervical spine, is reported in a 31-year-old man. The anomalies associated with these changes are described in detail in the text and illustrated radiologically. The relationship of the embryological alterations in contrast with the normal is described and emphasized, particularly in the upper cervical area. It is stressed that narrowing of the space from the back of the odontoid (or the posterior lip of the foramen magnum) is a direct result of the complex anomalies described in this case, producing compression of the medulla and/or the upper cervical spinal cord. The literature on this subject is reviewed in depth. (orig.)

  11. Early hemi-diaphragmatic plication following intraoperative phrenic nerve transection during complete AV canal repair

    Directory of Open Access Journals (Sweden)

    Hamad Alowayshiq

    2018-04-01

    Full Text Available Unilateral diaphragmatic palsy reduces pulmonary function by about 25% in older children and usually it is well tolerated; however, it causes severe respiratory distress in infants and young children. Diaphragmatic plication performed later than 10 days after cardiac surgery for patients under 1 year of age was associated with higher incidence of pneumonia and mortality. The management of the diaphragmatic paralysis due to phrenic nerve injury aiming mainly to preserve the respiratory function. Until now, the optimal management of diaphragmatic palsy in children who have undergone cardiac surgery remains controversial and consists of prolonged ventilation or diaphragmatic plication. In our case, many factors supported early diaphragmatic plication, the age of the patient, post-operative AV canal repair with severe pulmonary hypertension, and clear transection of the left phrenic nerve diagnosed intraoperatively.

  12. Ipsilateral Brachial Plexus Block and Hemidiaphragmatic Paresis as Adverse Effect of a High Thoracic Paravertebral Block

    NARCIS (Netherlands)

    Renes, Steven H.; van Geffen, Geert J.; Snoeren, Miranda M.; Gielen, Matthieu J.; Groen, Gerbrand J.

    Background: Thoracic paravertebral block is regularly used for unilateral chest and abdominal surgery and is associated with a low complication rate. Case Reports: We describe 2 patients with an ipsilateral brachial plexus block with Horner syndrome after a high continuous thoracic paravertebral

  13. Agenesia isolada da artéria pulmonar direita ou esquerda: avaliação da evolução natural e a longo prazo, após intervenção corretiva Isolated unilateral pulmonary artery agenesis: evaluation of natural and long term evolution after corrective surgery

    Directory of Open Access Journals (Sweden)

    Edmar Atik

    2006-10-01

    Full Text Available OBJETIVO: Agenesia da artéria pulmonar, direita ou esquerda, rara como anomalia isolada, foi relatada em 119 casos desde 1978. Apresenta-se clinicamente com hipertensão pulmonar (HP na infância e com hemoptise no adulto. Intervenções como reconstrução arterial pulmonar e lobectomia foram realizadas em 17% dos casos. Analisamos quatro destes casos, sendo dois em evolução natural e dois com regressão da HP, após longo tempo da correção operatória. MÉTODOS: Quatro crianças, três do sexo masculino, com 22,10 e 35 meses de idade e uma do sexo feminino com 20 meses, com insuficiência cardíaca direita (ICD e cianose na primeira e ICD na segunda e cansaço aos esforços nas outras duas. Todas tinham sinais de HP, sobrecarga ventricular direita no ECG e cardiomegalia. Cateterismo cardíaco mostrou pressões sistêmicas na artéria pulmonar contralateral na agenesia à direita em três e na agenesia à esquerda em um caso. RESULTADOS: Restabelecimento cirúrgico da continuidade arterial pulmonar foi possível em dois casos, na criança de 22 e na de 10 meses de idade, pela interposição de Goretex de diâmetro de 7 mm entre as artérias pulmonares até o hilo pulmonar contralateral hipoplásico. Houve regressão dos sinais de HP em período imediato e tardio, com 7 e 2,5 anos de idade respectivamente. A relação das pressões entre os ventrículos direito e esquerdo era de 30 e 40%, nos dois casos. A perfusão pulmonar aumentou de 8 para 44% e de 8 para 23%, nos dois casos. O mesmo procedimento foi programado para os outros pacientes. CONCLUSÃO: Esta técnica se torna a operação de escolha para casos similares, raramente descritos na literatura, mesmo em presença de acentuada HP e hipoplasia arterial pulmonar contralateral.OBJECTIVE: Unilateral pulmonary artery agenesis is an uncommon isolated anomaly and since 1978 only 119 cases have been reported. In general the condition presents as pulmonary hypertension (PH in children and

  14. Virtual Presenters: Towards Interactive Virtual Presentations

    NARCIS (Netherlands)

    Nijholt, Antinus; Cappellini, V.; Hemsley, J.

    2005-01-01

    We discuss having virtual presenters in virtual environments that present information to visitors of these environments. Some current research is surveyed and we will look in particular to our research in the context of a virtual meeting room where a virtual presenter uses speech, gestures, pointing

  15. Making your presentation fun: creative presentation techniques

    Energy Technology Data Exchange (ETDEWEB)

    KEENEN,MARTHA JANE

    2000-05-18

    What possesses someone to volunteer and go through hoops and red tape to make a presentation at a conference? For that matter, why does anyone ever present anything to anyone? Actually, presentations are a fact of life and there are many reasons for doing a presentation and doing it well. New and existing staff need training and orientation to the way things are done here. Handing all of them a manual and hoping they read it is pretty much a waste of paper. On the other hand, an effective, entertaining and upbeat presentation on the relevant topics is more likely to stick with those people. They will even have a name and face to remember and seek out when they have an issue on or with that topic. This can be a very effective beginning for networking with new peers. The presenter is seen as knowledgeable, as a source of information on company topics and possibly evaluated as a potential mentor or future manager. Project staff and/or peers benefit from clear, concise, presentations of topical knowledge. This is one way that a group working on various aspects of the same project or program can stay in touch and in step with each other. Most importantly, presentations may be the best or only door into the minds (and budgets) of management and customers. These presentations are a wonderful opportunity to address legal and compliance issues, budget, staffing, and services. Here is a chance, maybe the only one, to demonstrate and explain the wonderfulness of a program and the benefit they get by using the services offered most effectively. An interactive presentation on legal and compliance issues can be an effective tool in helping customers and/or management make good risk management decisions.

  16. Interim Restorative Approach for the Management of Congenitally Missing Permanent Mandibular Incisors: Presentation of Three Cases

    Directory of Open Access Journals (Sweden)

    Prashanth Prakash

    2011-01-01

    Full Text Available Congenital missing of mandibular permanent incisors with retained primary incisors may jeopardize the esthetic appearance and psychological development of children, especially during the years of transition into adolescence. The retained primary teeth are necessary for the maintenance and normal development of alveolar bone, which in turn is essential for future definitive rehabilitation. In such situations, an interim restoration may be provided before any definitive treatment is given to comfort the young patient during this transition period. Interim restorations may include resin-modified additions to the existing teeth as well as more sophisticated restorations such as resin-retained bridge and removable partial dentures. However, this restoration differs for different clinical situations based on various factors such as age and patient compliance, and also consideration has to be given for the growth changes of the child. The aim of this present paper is to discuss the esthetic management of three cases with bilateral agenesis of permanent mandibular incisors and retained primary incisors with composite interim restoration.

  17. Cognitive and behavioral functioning in Coffin-Siris syndrome and epilepsy: a case presentation.

    Science.gov (United States)

    Bender, H Allison; Zaroff, Charles M; Karantzoulis, Stella; Nakhutina, Luba; MacAllister, William S; Luciano, Daniel

    2011-01-01

    The authors characterized the cognitive, adaptive, and behavioral sequelae of Coffin-Siris (CS) syndrome and epilepsy in a 7.5-year-old child. Little is known about the early neurobehavioral presentation of CS. Clinical features consistent with this genetic anomaly include underdeveloped tips and nails of the fifth fingers, extended infranasal depression, and craniofacial abnormalities. MRI findings often reveal callosal agenesis. The authors conducted a neuropsychological evaluation and obtained parental ratings of behavioral and adaptive functioning. Attentional abilities were limited. As assessed by the Mullen Scales of Early Learning, receptive language abilities (age equivalent [AE]: 3-3) were relatively stronger than expressive skills (AE: 1-4). Adaptive functioning was low across all domains (Vineland Adaptive Behavior Composite AE: 1-9). On the Behavior Assessment for Children (BASC-2), social skills dysfunction, stereotyped and self-stimulatory behaviors, restricted interests, ritualistic play, and inappropriate object usage were noted. No significant mood disturbances were endorsed. Study findings indicate a diffuse pattern of neurobehavioral deficits in a child with CS and epilepsy. Further clinical assessment and research should include multidimensional assessment techniques, including evaluation of adaptive behavior, in an effort to capture the full range developmental sequelae in children with CS.

  18. Develop your presentation skills

    CERN Document Server

    Theobald, Theo

    2016-01-01

    Going beyond handling nerves and presenting PowerPoint slides, the third edition of "Develop Your Presentation Skills "offers practical advice on developing a captivating presentation, constructing compelling content, and boosting self-confidence. The book includes three new chapters on delivering a "stripped down"presentation, using new media to engage with the audience, and handling being asked to present on short notice."

  19. Congenital seminal vesicle cyst accompanying with ipsilateral renal agenesis in an adolescent patient: A pediatric radiologist approach to Zinner’s syndrome

    Directory of Open Access Journals (Sweden)

    Mehmet Burak Özkan

    2015-12-01

    Full Text Available A fifteen-year-old boy who had complaints of left sided pelvic pain with known ipsilateral left renal agenesia was referred to pediatric radiology department. Incidentally, his sonography examination revealed a dilated tubular structure located in the retro-vesicular region from cephalic to prostate. Contrast enhanced pelvic MRI showed a huge seminal vesicle cyst which is over 6 cm without a mass effect near the aspect border of the prostate and bladder. The patient was diagnosed with Zinner syndrome. The patient doesn’t have new complaint with no definite increase in the diameter of the cyst. In this case presentation we are discussing the Zinner syndrome’s imaging findings from a pediatric radiologist approach with a brief review of the literature.

  20. Subpart DD Training Presentations

    Science.gov (United States)

    EPA has produced the following training presentation(s) for reporters subject to this subpart. Generally, these presentations explain the rule or show how to use the reporting system e-GGRT to submit annual GHG reports to EPA.

  1. Subpart AA Training Presentations

    Science.gov (United States)

    EPA has produced the following training presentation(s) for reporters subject to this subpart. Generally, these presentations explain how to use the reporting system e-GGRT to submit annual GHG reports to EPA.

  2. Subpart W Training Presentations

    Science.gov (United States)

    EPA has produced the following training presentation(s) for reporters subject to this subpart. Generally, these presentations explain the rule or show how to use the reporting system e-GGRT to submit annual GHG reports to EPA.

  3. Rotating Poster Presentations

    Science.gov (United States)

    Lagares, Manuel; Reisenleutner, Sandra

    2017-01-01

    Oral presentations are a common practice in foreign language classes, often used to assess students' speaking skills. Usually, the presentations are delivered by students in front of the class, often with PowerPoint slides or Prezi as support. However, frequently the audience does not engage with the presentation and thus, the benefits of this…

  4. Presenting Food Science Effectively

    Science.gov (United States)

    Winter, Carl K.

    2016-01-01

    While the need to present food science information effectively is viewed as a critical competency for food scientists by the Institute of Food Technologists, most food scientists may not receive adequate training in this area. Effective presentations combine both scientific content and delivery mechanisms that demonstrate presenter enthusiasm for…

  5. Innovative presentations for dummies

    CERN Document Server

    Anthony, Ray

    2014-01-01

    Be the speaker they follow with breakthrough innovative presentations Innovative Presentations For Dummies is a practical guide to engaging your audience with superior, creative, and ultra-compelling presentations. Using clear language and a concise style, this book goes way beyond PowerPoint to enable you to reimagine, reinvent, and remake your presentations. Learn how to stimulate, capture, and hold your audience in the palm of your hand with sound, sight, and touch, and get up to speed on the latest presentation design methods that make you a speaker who gets audiences committed and acting

  6. Alarm filtering and presentation

    International Nuclear Information System (INIS)

    Bray, M.A.

    1989-01-01

    This paper discusses alarm filtering and presentation in the control room of nuclear and other process control plants. Alarm generation and presentation is widely recognized as a general process control problem. Alarm systems often fail to provide meaningful alarms to operators. Alarm generation and presentation is an area in which computer aiding is feasible and provides clear benefits. Therefore, researchers have developed several computerized alarm filtering and presentation approaches. This paper discusses problems associated with alarm generation and presentation. Approaches to improving the alarm situation and installation issues of alarm system improvements are discussed. The impact of artificial intelligence (AI) technology on alarm system improvements is assessed. (orig.)

  7. Behavioural present value

    OpenAIRE

    Krzysztof Piasecki

    2013-01-01

    Impact of chosen behavioural factors on imprecision of present value is discussed here. The formal model of behavioural present value is offered as a result of this discussion. Behavioural present value is described here by fuzzy set. These considerations were illustrated by means of extensive numerical case study. Finally there are shown that in proposed model the return rate is given, as a fuzzy probabilistic set.

  8. Conns' syndrome - atypical presentations

    International Nuclear Information System (INIS)

    Kumar, K V S Hari; Modi, K D; Jha, Sangeeta; Jha, Ratan

    2009-01-01

    Primary hyperaldosteronism (Conns' syndrome) commonly presents with a combination of clinical features of hypokalemia and hypertension. Atypical presentations like normotension, normokalemia and neurological ailments are described in few cases. We encountered two such cases, the first presenting with acute neurological complaint and second case having insignificant hypertension. Both the patients had a characteristic biochemical and imaging profile consistent with primary hyperaldosteronism and responded to surgical resection of adrenal adenoma. (author)

  9. CERN Electronics Pool presentations

    CERN Multimedia

    2011-01-01

    The CERN Electronics Pool has organised a series of presentations in collaboration with oscilloscope manufacturers. The last one will take place according to the schedule below.   Time will be available at the end of the presentation to discuss your personal needs. The Agilent presentation had to be postponed and will be organised later. -     Lecroy: Thursday, 24 November 2011, in 530-R-030, 14:00 to 16:30.

  10. Presentation skills for nurses.

    Science.gov (United States)

    Foulkes, Mark

    2015-02-20

    This article emphasises the importance of effective presentation skills. Such skills allow nurses to share knowledge and expertise and to communicate clearly in a range of workplace scenarios. Nurses are increasingly being asked to present in formal and informal situations, such as conferences, poster presentations, job interviews, case reports and ward-based teaching. This article explores the principles underpinning the development of these skills, discusses the situations in which they could be applied and demonstrates how nurses might improve and develop as presenters.

  11. Creating meaningful multimedia presentations

    NARCIS (Netherlands)

    Hardman, H.L.; Ossenbruggen, van J.R.

    2006-01-01

    Finding relevant information is one step in the chain of understanding information. Presenting material to a user in a suitable way is a further step. Our research focuses on using semantic annotations of multimedia elements to increase the "presentability" of retrieved information. We investigate

  12. Unusual presentation of sunburn.

    Science.gov (United States)

    Verma, Gopalkrishna G; Dave, Dhaval; Byrne, Eileen

    2008-10-01

    We present three cases of sunburn to the head, presenting with oedema of the face in children aged 6, 9 and 13 years. Oedema was predominantly on the forehead and temporal region; a direct effect of gravity was associated with erythema of the scalp. Sunburn healed without any complications.

  13. Presentation to the symposium

    International Nuclear Information System (INIS)

    Goodhead, D.

    1997-01-01

    A novel theory, the Second Event theory, advanced by Chris Busby, suggested that certain man-made radioisotopes could bypass cellular genetic repair mechanisms and cause cancer at very low dose. The present paper, after some introductory comments on the effects of particle tracks on cells, goes on to present the author's own perception of this Second Event theory. (UK)

  14. Conjugando el presente

    Directory of Open Access Journals (Sweden)

    Héctor Rojas Herazo

    1968-06-01

    Full Text Available La añoranza suele ser, casi siempre, la línea critica de menor resistencia. El lema de que "todo tiempo pasado fue mejor" tiene un poco la culpa. En cualquier orden del conocimiento el pasado sigue el verdadero presente. Ese presente en que nos gusta respirar y vivir. Nada de compromiso circundante. Nada de inmediatez.

  15. The presentation and treatment

    African Journals Online (AJOL)

    user

    tion of single collocations proposals are made for an implicit presentation of complex ... function lexicographers often include items to illustrate the typical use of the ... they have no items explicitly addressed at them to elevate them to treatment.

  16. PRESENT STATUS IN INDIA

    Indian Academy of Sciences (India)

    First page Back Continue Last page Overview Graphics. PRESENT STATUS IN INDIA. FIXED LINES – 36 MILLION. MOBILE CONNECTIONS – 14 MILLION. TELEDENSITY APPROXIMATELY 5. INTERNET CONNECTIONS – 5 MILLION. INTERNET USERS NEARLY – 25 MILLION.

  17. Fostering oral presentation performance

    NARCIS (Netherlands)

    Ginkel, van Stan; Gulikers, Judith; Biemans, Harm; Mulder, Martin

    2017-01-01

    Previous research revealed significant differences in the effectiveness of various feedback sources for encouraging students’ oral presentation performance. While former studies emphasised the superiority of teacher feedback, it remains unclear whether the quality of feedback actually differs

  18. Present status of dibaryons

    International Nuclear Information System (INIS)

    Locher, M.P.

    1981-01-01

    In the present notes the author tries to assess the situation regarding dibaryons in the various channels with baryon number two, emphasizing the problems and discussing in greater detail the most recent developments since the Versailles Conference. One of the motivations for the present search of B=2 states is the possibility of colored subclusters for systems with six quarks which cannot occur for ordinary baryons or mesons. (Auth.)

  19. Presenting New Grammar

    Institute of Scientific and Technical Information of China (English)

    WU Cai-ling; WANG Xi

    2015-01-01

    More and more researchers have now agreed upon the necessity of teaching grammar, but it still remains controversial as how to teach the forms, with the central consideration of not to harm the meaning-focused communicative teaching method. In this essay, one of the issues in grammar teaching will be discussed as how to present new grammar to learners, through evaluating and modifying a particular presentation activity in a grammar-teaching textbook.

  20. Stellar Presentations (Abstract)

    Science.gov (United States)

    Young, D.

    2015-12-01

    (Abstract only) The AAVSO is in the process of expanding its education, outreach and speakers bureau program. powerpoint presentations prepared for specific target audiences such as AAVSO members, educators, students, the general public, and Science Olympiad teams, coaches, event supervisors, and state directors will be available online for members to use. The presentations range from specific and general content relating to stellar evolution and variable stars to specific activities for a workshop environment. A presentation—even with a general topic—that works for high school students will not work for educators, Science Olympiad teams, or the general public. Each audience is unique and requires a different approach. The current environment necessitates presentations that are captivating for a younger generation that is embedded in a highly visual and sound-bite world of social media, twitter and U-Tube, and mobile devices. For educators, presentations and workshops for themselves and their students must support the Next Generation Science Standards (NGSS), the Common Core Content Standards, and the Science Technology, Engineering and Mathematics (STEM) initiative. Current best practices for developing relevant and engaging powerpoint presentations to deliver information to a variety of targeted audiences will be presented along with several examples.

  1. Paediatrics brain imaging in epilepsy: common presenting symptoms and spectrum of abnormalities detected on MRI

    International Nuclear Information System (INIS)

    Ali, A.; Akram, F.; Khan, G.; Hussain, S.

    2017-01-01

    Epilepsy, a common neurological disorder can present at any age and has a number of aetiologies with underlying brain disease being the most common aetiology. Brain imaging becomes important and mandatory in the work up for epilepsy in localization and lateralization of the seizure focus. Methods: This cross-sectional study was conducted in the department of Radiology Ayub Medical Teaching Institution Abbottabad from 1st March 2015 to 31st March 2016. A total of 209 children aged 28 days to 14 years were included in the study who presented with seizures to clinicians. Information obtained from history, clinical examination and investigations especially MRI brains were recorded in a prescribed pro forma. The data was analysed in SPSS 20. Results: MRI examination was unremarkable in 44.01% (n=92) and mild generalized brain atrophy was noted in 12.91% (n=27). Arachnoid cysts, mild unilateral brain atrophy and hydrocephalous due to aqueduct stenosis were recorded in 3.82% (n=8) of each group. Neoplastic lesions were the second most common abnormal MRI finding and constituted 5.74% (n=12). Leukodystrophy was diagnosed in 4.78% (n=10). MRI examination showed ring enhancing lesions (tuberculomas) and AVM in 1.43% (n=3) of each group. Perinatal ischemia and intracranial infection, (focal or generalized) were recorded in 2.87% (n=6) of each group. A 0.95 % (n=2) of children in each group had agenesis of corpus callosum and cavernoma. The radiological MRI diagnosis of Raussmussen encephalitis was made in 3.34% (n=7). Single case, each of mesial temporal sclerosis, subdural haemorrhage, infarct and craniopharyngioma was recorded making 0.47 % of the total patients in each case. Conclusion: MRI examination was abnormal in significant number of patients (55.86%), so therefore if properly utilized, in a good clinical context, this can identify most of the structural brain abnormalities in paediatric patients presenting with seizures. (author)

  2. Milk: Past and Present

    Science.gov (United States)

    Bulajić, S.; Đorđević, J.; Ledina, T.; Šarčević, D.; Baltić, M. Ž.

    2017-09-01

    Although milk/dairy consumption is part of many cultures and is recommended in most dietary guidelines around the world, its contribution to overall diet quality remains a matter of controversy, leading to a highly polarized debate within the scientific community, media and public sector. The present article, at first, describes the evolutionary roots of milk consumption, then reviews the milk-derived bioactive peptides as health-promoting components. The third part of the article, in general, presents the associations between milk nutrients, disease prevention, and health promotion.

  3. Book Presentation | 27 February

    CERN Multimedia

    The CERN Library

    2013-01-01

    "A caccia del bosone di Higgs. Magneti, governi, scienziati e particelle nell'impresa scientifica del secolo" by Luciano Maiani and Romeo Bassoli, published by Mondadori. On Wednesday 27 February Luciano Maiani will present the book "A caccia del bosone di Higgs. Magneti, governi, scienziati e particelle nell'impresa scientifica del secolo". More information here. The presentation will take place in the Council Chamber at 16.00 and will be followed by a debate and book signing. The book will be on sale. You are cordially invited.

  4. Renewable Electricity Futures (Presentation)

    Energy Technology Data Exchange (ETDEWEB)

    Mai, T.

    2012-08-01

    This presentation library summarizes findings of NREL's Renewable Electricity Futures study, published in June 2012. RE Futures investigated the challenges and impacts of achieving very high renewable electricity generation levels in the contiguous United States by 2050. It was presented at the 2012 RE AMP Annual Meeting. RE-AMP is an active network of 144 nonprofits and foundations across eight Midwestern states working on climate change and energy policy with the goal of reducing global warming pollution economy-wide 80% by 2050.

  5. Hera presentation generator (Poster)

    NARCIS (Netherlands)

    Frasincar, F.; Houben, G.J.P.M.; Barna, P.; Ellis, A.; Hagino, T.

    2005-01-01

    Semantic Web Information Systems (SWIS) are Web Information Systems that use Semantic Web technologies. Hera is a model-driven design methodology for SWIS. In Hera, models are represented in RDFS and model instances in RDF. The Hera Presentation Generator (HPG) is an integrated development

  6. Creating Meaningful Multimedia Presentations

    NARCIS (Netherlands)

    L. Hardman (Lynda); J.R. van Ossenbruggen (Jacco)

    2006-01-01

    textabstractFinding relevant information is one step in the chain of understanding information. Presenting material to a user in a suitable way is a further step. Our research focuses on using semantic annotations of multimedia elements to increase the ”presentability” of

  7. AECL present and future

    International Nuclear Information System (INIS)

    Foster, J.S.

    The history and present activities of the Crown corporation AECL are described. AECL owns limited interests in nuclear power plants and a Manitoba transmission system. AECL is the world's foremost producer of heavy water, and exports CANDU type reactors and radiation processing equipment. AECL backs up the Canadian nuclear power program wherever there is no conflict with private enterprise. (E.C.B.)

  8. Econophysics: Past and present

    Science.gov (United States)

    de Area Leão Pereira, Eder Johnson; da Silva, Marcus Fernandes; Pereira, H. B. B.

    2017-05-01

    This paper provides a brief historical review of the relationship between economics and physics, beginning with Adam Smith being influenced by Isaac Newton's ideas up to the present day including the new econophysics discipline and some of the tools applied to the economy. Thus, this work is expected to motivate new researchers who are interested in this new discipline.

  9. Present State Examination

    DEFF Research Database (Denmark)

    Pedersen, Kamilla; Bertelsen, Aksel

    Present State Examination (PSE) 5. udgave er en revideret version af speciallæge i psykiatri Aksel Bertelsens oprindelige materiale. Aksel Bertelsen er ophavsmand til PSE og til den danske udgave af ICD-10. Revisionen omfatter et forbedret layout, en modernisering af sproget, tilføjelse af...

  10. Presentation of CEA

    International Nuclear Information System (INIS)

    Graf, J.J.

    1976-01-01

    The organization of the CEA, its missions and means are presented. Its activities in the field of light water reactors, fast neutron reactors and PWR reactors of small and medium power for electricity and/or heat generation are emphasized [fr

  11. Going beyond methodological presentism

    DEFF Research Database (Denmark)

    Schmidt, Garbi

    2017-01-01

    Denmark is an example of a country where the idea of historical ethnic homogeneity stands strong. This article challenges this historical presentism: the scholarly and societal tendency to understand social phenomena within a limited contemporary framework, neglecting possible effects and similar...

  12. Analyzing in the present

    DEFF Research Database (Denmark)

    Revsbæk, Line; Tanggaard, Lene

    2015-01-01

    The article presents a notion of “analyzing in the present” as a source of inspiration in analyzing qualitative research materials. The term emerged from extensive listening to interview recordings during everyday commuting to university campus. Paying attention to the way different parts of vari...

  13. Presentation on ecoenergetic assessment

    International Nuclear Information System (INIS)

    Fournelle, C.

    1998-01-01

    The development of a tool to assess the quality of indoor air for buildings in the residential and commercial sector was presented. The computer software program was designed to allow a building operator to set an optimal target to reflect the greatest economies in energy consumption. Air quality was assessed in terms of carbon dioxide, volatile organic compounds, and other common indoor pollutants

  14. Keynote presentation : SAR systems

    NARCIS (Netherlands)

    Halsema, D. van; Otten, M.P.G.; Maas, A.P.M.; Bolt, R.J.; Anitori, L.

    2011-01-01

    Synthetic Aperture Radar (SAR) systems are becoming increasingly important sensors in as well the military environment as in the civilian market. In this keynote presentation an overview will be given over more than 2 decades of SAR system∼ and SAR application development at TNO in the Netherlands.

  15. Agriculture Oral Presentations

    International Nuclear Information System (INIS)

    1997-01-01

    This publication contains 23 papers related to the use of nuclear techniques in plant breeding in Turkey, effect of gamma irradiations on growing various plants, mutations and soil chemistry, etc., presented at 4. International Congress of Nuclear Agriculture and Animal Science in Bursa, Turkey, 25-27 Sep 1996. A separate abstract was prepared for each paper

  16. Imagined futures, present lives

    DEFF Research Database (Denmark)

    Dalsgaard, Anne Line; Wildermuth, Norbert

    2006-01-01

    is discussed centrally in this attempt to contribute to an empirically grounded understanding of the role that media play for youth in their striving to ‘find a place in life'. In the empirical context presented in the article, imaginations, expanded and circulated by a globalized media circuit...

  17. Presentation of 2004 earnings

    International Nuclear Information System (INIS)

    2005-03-01

    This presentation offers financial information on the AREVA Group earnings for the year 2004. With manufacturing facilities in over 40 countries and a sales network in over 100, Areva offers customers technological solutions for nuclear power generation and electricity transmission and distribution. The Group also provides interconnect systems to the telecommunications, computer and automotive markets. (A.L.B.)

  18. Summary of the presentations

    International Nuclear Information System (INIS)

    2002-01-01

    This part summarizes the content of the six following presentations: 1. IAEA requirements/guidance for the decommissioning safety case (Mike Bell, IAEA); 2. Safety case in an EIA context (Patrick O'SULLIVAN, The Netherlands); 3. Plant configuration and the D and D licensing process (Eugenio GIL LOPEZ and Pedro CARBONERAS, Spain); 4. Accident assessment (Ivo TRIPPUTI, Italy; Ingmar LUND, Sweden); 5. Balancing radiological and industrial risk in the safety case (Pascal DEBOODT, Belgium); 6. The safety case for safe store and dormancy periods (Paul WOOLLAM, UK). Following the six presentations, the WPDD members discussed the safety case in an open discussion led by the Topical Session Chair, Frances Taylor. The discussion was wide-ranging, and topics varied from the standards that apply to releases to the environment during safe store, to decommissioning parallels with other major processing industries and facilities (such as oil rigs). Frances Taylor steered the discussion toward addressing the questions that the WPDD Core Group had proposed for the topical session. The Chair, the Rapporteur, and some members of the group sensed that the presentations had covered such an wide variety of issues, and that the content of the presentations themselves differed so greatly, that it would be difficult to distill a position paper from the information gathered, as had been the mandate from the Core Group. In addition, the meaning of the term 'Safety Case' differed greatly among participants in the topical session. Some took the term to mean the analysis and document that the implementor provides to the regulator, describing how operations will be performed safely. Others considered the term to mean the overall safety and risk conditions inherent in a nuclear facility during operations or decommissioning. With the differences in presentations and with the meaning of the terminology remaining unclear, the WPDD participants did not reach consensus conclusions on December 5, 2001

  19. [Metachromatic Leukodystrophy. Case Presentation].

    Science.gov (United States)

    Espejo, Lina María; de la Espriella, Ricardo; Hernández, José Fernando

    Metachromatic leukodystrophy (MLD) is a rare demyelinating disease (prevalence 1:40 000), also called arylsulfatase A deficiency (ARS-A), which may present with neurological and psychiatric symptoms. Clinical assessment may be difficult, due to unspecific signs and symptoms. A case is presented of a 16 year-old female patient seen in psychiatry due to behavioural changes, psychosis, and with impaired overall performance. She was initially diagnosed with schizophrenia, but the Nuclear Magnetic Resonance (NMR) scan and laboratory tests lead to the diagnosis of MLD. Copyright © 2016 Asociación Colombiana de Psiquiatría. Publicado por Elsevier España. All rights reserved.

  20. Organising and presenting information.

    Science.gov (United States)

    Kankanady, Raghavendra; Wells, Marilyn

    2013-01-01

    Information management can be a daunting process for clinicians, health care providers and policy makers within the health care industry. This chapter discusses the importance of information classification and information architecture in the information economy and specific challenges faced within the health care industry. The healthcare sector has industry specific requirements for information management, standards and specifications for information presentation. Classification of information based on information criticality and the value in the health care industry is discussed in this paper. Presentation of information with reference to eHealth standards and specifications for healthcare information systems and their key requirements are also discussed, as are information architecture for eHealth implementation in Australia. This chapter also touches on information management and clinical governance since the importance of information governance is discussed by various researchers and how this is becoming of value to healthcare information management.

  1. Atypical Presentations of Tularemia.

    Science.gov (United States)

    Odegaard, Karah; Boersma, Beth; Keegan, James

    2017-05-01

    Francisella tularensis is a gram-negative coccobacillus that causes a condition commonly referred to as tularemia. There has been a dramatic increase in tularemia cases reported in South Dakota, many of which were challenging to diagnose due to atypical clinical manifestations. We describe an interesting case of pneumonic tularemia and summarize six similar cases, several of which presented with lung nodules suggestive of malignancy. According to the literature, this is only the third outbreak of pneumonic tularemia reported in the U.S. We believe it is important for clinicians to be aware of the increased incidence of tularemia in the area and to be vigilant in the diagnosis and management of these atypically presenting cases. Copyright© South Dakota State Medical Association.

  2. Presentation = Speech + Slides

    Directory of Open Access Journals (Sweden)

    Derik Badman

    2008-12-01

    Full Text Available Back in October, Aaron Schmidt posted “HOWTO give a good presentation” to his blog walking paper. His second bullet point of “thoughts” on good presentations is: Please don’t fill your slides with words. Find some relevant and pretty pictures to support what you’re saying. You can use the pictures to remind yourself what you’re going [...

  3. EPMA present and future

    International Nuclear Information System (INIS)

    Love, G.

    2002-01-01

    In electron probe microanalysis the objective has always been to gain the maximum information from a sample. To this end efforts have been directed at increasing detection efficiency, reducing the size of the excitation volume, analysing more difficult specimens and improving the accuracy of quantitative measurements whilst, at the same time, attempting to minimize the complexity of the analysis. The present paper comments on the progress that has been made in this areas, focuses on the latest hardware and software developments taking place and looks forward to how these may impact on the technique of EPMA. (author)

  4. Sarcoidosis Presenting Addison's Disease.

    Science.gov (United States)

    Takahashi, Kentaro; Kagami, Shin-Ichiro; Kawashima, Hirotoshi; Kashiwakuma, Daisuke; Suzuki, Yoshio; Iwamoto, Itsuo

    2016-01-01

    We herein describe a second Japanese case of sarcoidosis presenting Addison's disease. A 52-year-old man was diagnosed with sarcoidosis based on clinical and laboratory findings, including bilateral hilar lymphadenopathy and elevated levels of serum angiotensin-converting enzyme and lysozyme, as well as the presence of noncaseating epithelioid granulomas. The patient also exhibited general fatigue, pigmentation, weight loss, hypotension and hyponatremia, suggestive of chronic adrenocortical insufficiency. An endocrine examination confirmed primary adrenocortical insufficiency. This case suggests the direct involvement of sarcoid granuloma in the adrenal glands.

  5. Radwaste - Multimedia presentation

    International Nuclear Information System (INIS)

    2000-01-01

    Radioactivity is a part of the Nature since the creation of the Earth. We have learned how to use radioactivity in medicine, power industry, and in other areas of life. Similarly as in other activities, waste is generated. We offer you basic information about radioactivity and radioactive waste (radwaste) in four films: Radioactivity, Source of radwaste, Management of radwaste. In the glossary are video presentations: Container (Castor) for radioactive waste; Deep underground repository; Transport vehicle; WWER type reactor. Encyclopedic part of the program contains detailed theoretical and practical information about use of radioactivity and radioactive waste. You will find the explanation in the part Glossary. (authors)

  6. Presenting Newtonian gravitation

    International Nuclear Information System (INIS)

    Counihan, Martin

    2007-01-01

    The basic principles of the Newtonian theory of gravitation are presented in a way which students may find more logically coherent, mathematically accessible and physically interesting than other approaches. After giving relatively simple derivations of the circular hodograph and the elliptical orbit from the inverse-square law, the concept of gravitational energy is developed from vector calculus. It is argued that the energy density of a gravitational field may reasonably be regarded as -g 2 /8πG, and that the inverse-square law may be replaced by a Schwarzschild-like force law without the need to invoke non-Euclidean geometry

  7. Presentation to the symposium

    International Nuclear Information System (INIS)

    Busby, C.

    1997-01-01

    This paper proposes that there is a major error in the present perception of cancer risk from exposure to low level radiation from internal man-made fission products like strontium-90. The author's Second Event theory is discussed which advances the basis that fission products are different in radiation risk terms. Since the scientific community has been so ready to dismiss this idea out of hand, the author also explores scientific culture and scientific revolutions. The evidence that the idea is broadly right is surveyed and finally some studies that could be undertaken to prove or disprove the author's contention are outlined. (UK)

  8. Radiation myelopathy (case presentation)

    International Nuclear Information System (INIS)

    Sangheli, M.; Lisnic, V.; Plesca, S.; Odainic, O.; Chetrari, L.

    2009-01-01

    The radiotherapy is one of the most widely spread and commonly used method in treating tumors of different localization. A detailed analysis of benefits and possible side effects along with evaluation of the risk factors allows preventing one of the most difficult complication, and namely the radiation myelopathy. The delayed form of such a disease is distinguished by a pronounced unfavorable prognosis. The presented case provoked the discussion of difficulties in performing differential diagnosis, as well as the importance of determining the localization of vulnerable tissues as regards the target organ exposed to radiotherapy. (authors)

  9. DOE financial assurance presentation

    International Nuclear Information System (INIS)

    Huck, R.

    1990-01-01

    The presentation topic is California's approach to license application review in meeting financial assurances for the proposed Ward Valley site. The purpose of the presentation is to provide information on specific financial assurance provisions contained in 10 CFR Part 61 and how California intends to satisfy those requirements. Also, as rate setter, California intends to demonstrate how it will assure allowable costs to the rate base though a financial prudency review. The key provisions of financial assurance are: 10 CFR Section 61.61 - This provision requires an applicant to demonstrate its ability to finance licensed activities; 10 CFR Section 61.62 - This provision requires an applicant to provide assurance that sufficient funds will be available for site closure and stabilization; and 10 CFR Section 61.63 - This provision requires an applicant to provide 'a copy of a binding arrangement, such as a lease, between the applicant and the disposal site owner, so that sufficient funds will be available to cover the costs of the institutional control period.' To assist California in its determination of financial assurance compliance to be demonstrated by the applicant for Part 61 requirements, is NUREG guidance document 1199 'Standard Format and Content of a License Application for a Low-Level Radioactive Waste (LLRW) Disposal Facility.' The detailed financial assurance provisions of NUREG 1199 are then embodied in NUREG 1200, 'Standard Review Plant for the Review of a License Application for a LLRW Disposal Facility.'

  10. Rett syndrome: EEG presentation.

    Science.gov (United States)

    Robertson, R; Langill, L; Wong, P K; Ho, H H

    1988-11-01

    Rett syndrome, a degenerative neurological disorder of girls, has a classical presentation and typical EEG findings. The electroencephalograms (EEGs) of 7 girls whose records have been followed from the onset of symptoms to the age of 5 or more are presented. These findings are tabulated with the Clinical Staging System of Hagberg and Witt-Engerström (1986). The records show a progressive deterioration in background rhythms in waking and sleep. The abnormalities of the background activity may only become evident at 4-5 years of age or during stage 2--the Rapid Destructive Stage. The marked contrast between waking and sleep background may not occur until stage 3--the Pseudostationary Stage. In essence EEG changes appear to lag behind clinical symptomatology by 1-3 years. An unexpected, but frequent, abnormality was central spikes seen in 5 of 7 girls. They appeared to be age related and could be evoked by tactile stimulation in 2 patients. We hypothesize that the prominent 'hand washing' mannerism may be self-stimulating and related to the appearance of central spike discharges.

  11. Bad (Re)Presentations

    CERN Multimedia

    Computer Security Team

    2013-01-01

    This time, we would like to address copyright violations (again) - but with a twist. While previous articles focussed on “Music, videos and the risk for CERN” or on software licences, the focus is now on using photos, music and images “found” on the Web for presentations, publications and web pages…   Just recently, a video produced for CERN and published on YouTube and Facebook used background music from a contemporary popular artist. However, the people responsible failed to obtain the appropriate permission to actually use that music. They thought that having bought it on iTunes was sufficient. But it was not: “buying” means you have the right to listen, but not the right for further distribution or re-publication. As a result, the videos were pulled from YouTube and Facebook. Similarly, how many of us integrate graphics and images “found” on the Web into our presentations?  How many of us...

  12. Film Presentation: Die Urknallmaschine

    CERN Multimedia

    Carolyn Lee

    2010-01-01

    Die Urknallmaschine, an Austrian film by Gerd Baldauf, narrated by Norbert Frischauf (Alpha Österreich - ORF, 2009).  In CERN’s gigantic complex particles are accelerated to almost the speed of light, brought to collision and made to divide into even smaller particles. Public opinion of CERN’s research is also divided. Sceptics fear that black holes may be created. Might the goal to study the origin of the world lead to its destruction? The Austrian researcher Norbert Frischauf worked at CERN for many years. With his guidance it is possible to explore the world’s largest research centre, get a glimpse of the fascinating work the scientists do there and take a crash course in particle physics. Die Urknallmaschine will be presented on Friday, 25 June from 13:00 to 14:00 in the Main Auditorium. Language: German

  13. Abdominal pregnancy - Case presentation.

    Science.gov (United States)

    Bohiltea, R; Radoi, V; Tufan, C; Horhoianu, I A; Bohiltea, C

    2015-01-01

    Abdominal pregnancy, a rare diagnosis, belongs to the ectopic pregnancy group, the leading cause of pregnancy related exitus. The positive diagnosis is very difficult to establish most often in an acute setting, leading to a staggering percent of feto-maternal morbidity and mortality. We present the case of 26-weeks-old abdominal pregnancy with partial feto-placental detachment in a patient, after hysteroscopy and in vitro fertilization, which until the acute symptoms that led to emergency laparotomy went unrecognized. The patient recovered completely and satisfactorily after surgery and, due to the high risk of uterine rupture with regard to a second pregnancy, opted for a surrogate mother. Abdominal pregnancy can be regarded as a difficult to establish diagnosis, with a greater chance in case of increased awareness. It is compulsory to be well informed in order not to be surprised by the diagnosis and to apply the correct treatment immediately as the morbidity and mortality rate is elevated.

  14. Trichinosis - case presentation

    Directory of Open Access Journals (Sweden)

    Rajko Saletinger

    2007-03-01

    Full Text Available Background: Trichinosis is a widespread zoonosis acquired by ingestion of undercooked meat infected with the larvae of Trichinella parasites. The disease has variable clinical manifestations ranging from asymptomatic to fatal. Majority of infections is caused by Trichinella spiralis, although there are some cases caused by other species. The dramatic decline of trichinosis reflects changes in industrial practices and increased public awareness. The majority of human cases are now associated with wild game meat and noncommercial pork. The case of previously healthy, young man with trichinosis is presented. The disease was determined with serologic testing and cured with albendazole. The source of infection has not been determined.Conclusions: In patients with fever, mialgias and eosinophilia infection with tissue nematodes should always be taken in consideration. In those who have recently consumed raw or undercooked pork and wild game meat we should think about trichinosis. The disease is rare in Slovenia.

  15. Imagined futures, present lives

    DEFF Research Database (Denmark)

    Dalsgaard, Anne Line; Wildermuth, Norbert

    2006-01-01

    The article is focused thematically on the uses and meanings of media for (some) young people in Recife, a million-inhabitant city in the northeast of Brazil. The article brings together the perspective of Anne Line Dalsgaard, a long-term anthropological field researcher who is familiar with the ......The article is focused thematically on the uses and meanings of media for (some) young people in Recife, a million-inhabitant city in the northeast of Brazil. The article brings together the perspective of Anne Line Dalsgaard, a long-term anthropological field researcher who is familiar...... is discussed centrally in this attempt to contribute to an empirically grounded understanding of the role that media play for youth in their striving to ‘find a place in life'. In the empirical context presented in the article, imaginations, expanded and circulated by a globalized media circuit...

  16. Presenting practice financial information.

    Science.gov (United States)

    Webster, Lee Ann H

    2007-01-01

    Medical practice leadership teams, often consisting primarily of physicians with limited financial backgrounds, must make important business decisions and continuously monitor practice operations. In order to competently perform this duty, they need financial reports that are relevant and easy to understand. This article explores financial reporting and decision-making in a physician practice. It discusses reports and tools, such as ratios, graphs, and comparisons, that practices typically include in their reports. Because profitability and cash flow are often the most important financial considerations for physician practices, reports should generally focus on the impact of various activities and potential decisions upon these concerns. This article also provides communication tips for both those presenting practice financial information and those making the decisions. By communicating effectively, these leaders can best use financial information to improve decision-making and maximize financial performance.

  17. Presentation of Canada

    International Nuclear Information System (INIS)

    Hedley, Dianne E.

    1997-01-01

    In contingency of a nuclear emergency event, requiring application of intervention measures on a federal scale, Canada has of a plan ensuring the compatibility of the plans of different provinces and serving as interface between federal and provincial authorities. Exclusive of a nuclear attack against North America, by nuclear emergency it is understood an accident resulting in radionuclide release. This is called the Plan of federal intervention in case of nuclear emergency. 'Sante Canada' is the federal authority responsible for intervention in case of nuclear emergency and it has the task of preparing and coordinating the actions on a federal scale.Should the plan be set in action and if the emergency has repercussions upon the agricultural sector, the sustaining organism will be 'Agriculture and agroalimentaire Canada' which in case of emergency acts through the channels of the National System of intervention in the agro-alimentary sector (SNIUA). The paper presents the objectives, the principles of organization and operation, the responsibilities and the plans which SNIUA has in its charge to implement in case of emergency

  18. STS-69 postflight presentation

    Science.gov (United States)

    1995-10-01

    A postflight conference of the STS-69 mission is presented. The flightcrew ('The Dog Team') consisted of Cmdr. David Walker, Pilot Kenneth Cockrell, Payload Cmdr. James Voss, and Mission Specialists James Newman and Michael Gernhardt. The mission's primary objective was the deployment and retrieval of the SPARTAN-201 satellite, which investigated the interaction between the Sun and it's solar wind. Other secondary experiments and shuttle payloads included the Wake Shield Facility (WSF), which grew several layers of semiconductor films, the International Extreme Ultraviolet Hitchhiker (IEH-1), the Capillary Pumped Loop-2/Gas Bridge Assembly (CAPL-2/GBA), several Get Away Specials (GAS) experiments, the Electrolysis Performance Improvement Concept Study (EPICS), the Thermal Energy Storage (TES-2) experiment, the Commercial Generic Bioprocessing Apparatus-7 (CGBA-7), the National Institutes of Health-Cells 4 (NIH-C4) experiment, and the Biological Research in Canister-6 (BRIC-6) experiment. Earth views consisted of Saudi Arabia water wells, uncommon vortices over Oman, the Amazon River, the Bahamas, Somalia, a sunset over the Earth's horizon, and two hurricanes, Luis and Marilyn.

  19. Present energy status

    Energy Technology Data Exchange (ETDEWEB)

    Desprairies, P

    1988-04-01

    This paper reviews the past and present situation of the World Oil Market and assumes future trends and possible future evolution. Low oil prices between 1950 and 1973 have contributed to an exceptional growth of oil demand during more than 20 years which was more and more supplied by OPEC. The oil shocks of 1973 and 1979 have dramatically changed this situation. Some of the most important changes have been the emergence of non-OPEC new oil producers, the growth of substitute energies, especially natural gas and nuclear power, on the supply side; and substantial decrease of energy consumption in many industrialized countries which has greatly reduced oil demand to OPEC on the demand side. Today this situation seems somewhat stabilized (after the reversal of world oil prices in 1986) and OPEC is producing around 18 million b/day as against a producing capacity of about 30 million b/day. It is generally considered that in coming years oil prices will remain relatively low (or occasionally even become lower than today) due to excess oil production capacity and relative abundance of other types of energy. However, many experts think that these trends can be reversed when oil supply will more and more concentrate again in the Middle East; but it is hard to say if this will begin in 1995, 2000, 2010.

  20. The laryngocele; case presentation

    Directory of Open Access Journals (Sweden)

    Gabriel V. Berteșteanu

    2016-05-01

    Full Text Available Laryngocele is a rare pathology, but because of their clinical evolution and the symptoms they generate, they should always be considered as a differential diagnosis when investigating neck masses. A laryngocele is basically a herniation of the mucosa of the laryngeal ventricle (Morgagni's ventricle arising usually from the saccular region. This herniation may remain confined to the larynx - in which case the laryngocele is internal- or expand through the thyro-hyoid membrane into the structures of the neck - thus being called an external laryngocele. Usually the laryngocele has both an internal and external component thus being a mixed laryngocele. Diagnosis of laryngoceles still relies heavily on clinical signs such as tympanism, easily depressible neck mass, indirect laryngoscopy, but is now simplified by imagistic investigations (ultrasound, CT and MRI. However, the treatment of this condition is exclusively surgical and consists of total excision of the laryngocele, as well as proper identification of the point of origin from the saccule and also the final suture of the breach in order to prevent recurrence. Investigation of possible causes of obstruction of the laryngeal ventricle should always be performed (because of the possibility of an underlying malignancy as well as a follow-up protocol of the patient, given the risk of relapse. We present a recently diagnosed case of a 32 year old man with mixed laryngocele, which we have operated in our clinic.

  1. [Antibiotics: present and future].

    Science.gov (United States)

    Bérdy, János

    2013-04-14

    The author discuss the up to date interpretation of the concept of antibiotics and antibiotic research, as well as the present role of various natural, semisynthetic and synthetic antibiotic compounds in various areas of the human therapy. The origin and the total number of all antibiotics and applied antibiotics in the practice, as well as the bioactive microbial metabolites (antibiotics) in other therapeutical, non-antibiotic fields (including agriculture) are also reviewed. The author discusses main problems, such as increasing (poly)resistance, virulence of pathogens and the non-scientific factors (such as a decline of research efforts and their sociological, economic, financial and regulatory reasons). A short summary of the history of Hungarian antibiotic research is also provided. The author briefly discusses the prospects in the future and the general advantages of the natural products over synthetic compounds. It is concluded that new approaches for the investigation of the unlimited possibilities of the living world are necessary. The discovery of new types or simply neglected (micro)organisms and their biosynthetic capabilities, the introduction of new biotechnological and genetic methods (genomics, metagenom, genome mining) are absolutely required in the future.

  2. IKONOS: future and present

    Science.gov (United States)

    Schaap, Niek

    2003-04-01

    The IKONOS satellite has been operational since January 2000 and was the first commercial satellite collecting imagery with 1 meter resolution. The current life expectancy of the satellite is 10 years. Since the launch, Space Imaging Inc. (the owner of the satellite) supplied IKONOS imagery to users in many vertical markets, such as: agriculture, defense, oil & gas and telecommunications. This oral presentation will give comprehensive information about IKONOS and the future: * Block II, the successor of IKONOS. Space Imaging expects to launch in 2004 a new high-resolution satellite, ensuring both continuity and (for some years) a tandem operation with IKONOS, greatly improving the availability of imagery. * Space Imaging affiliates. IKONOS imagery collected, processed and sold by regional affiliates. These regional affiliates are strategically located around the world, like Japan Space Imaging (Tokyo), Space Imaging Middle East (Dubai) and Space Imaging Eurasia (Ankara, Turkey). * Technical briefing IKONOS. IKONOS (compared to other commercial high-resolution satellites) has superior collection capabilities. Due to, the higher orbit altitude, local reception of the imagery, bi-directional scanning and the high agility of the satellite, is the IKONOS satellite capable to collect the imagery relative quickly.

  3. Presentation of Pydio

    CERN Multimedia

    CERN. Geneva

    2014-01-01

    **Pydio is an open-source platform for file sharing and synchronisation.** It is wildly used by enterprises and organizations worldwide, including major universities. Pydio comes with a rich web-based interface, native clients for both iOS and Android mobile devices, and lately a new sync client for desktop platforms. Pydio v6 is of great useability and introduces many new features that makes it the ultimate sharing machine. Its architecture makes it a perfect fit for either easily deploying an on-premise dropbox solution, or building more complicated solutions where the box feature is integrated as OEM. Storage-agnostic, Pydio is a simple layer that can be deployed on top of any storage backend, thus providing scalability and high-availability out-of-the-box. Charles du Jeu is the lead developer of the solution and will briefly present its feature and how it can fit for research and engineering purpose. See https://pyd.io/ for more information.

  4. THE UPSIDE OF PRESENTISM

    Directory of Open Access Journals (Sweden)

    Lynn Fendler

    2009-01-01

    Full Text Available El presentismo es visto generalmente como un mal necesario en historiografía. Este artículo explora lo positivo de esta inevitabilidad. Aplicando un enfoque filosófico al análisis discursivo en la tradición de la nueva historia cultural, se distingue -de una parte-¿ entre un uso estratégico del presentismo, y -de otra- un enfoque racionalista en historia. El artículo concluye considerando algunas implicaciones políticas en historiografía de las explicaciones racionalistas y de las explicaciones estratégicamente presentistas. Los modos de comprensión racionalistas inscriben las expectativas del pasado en las visiones del futuro; asignando al historiador el papel de profeta; y perpetuando nociones de agentes ahistóricos. En contraste, las historias estratégicamente presentistas incorporan una orientación que deliberadamente emplea las lentes y perspectivas del presente en orden a tener en cuenta supuestos y perspectivas actuales. Cuando las asunciones son examinadas en relación a las perspectivas presentistas, dichos presupuestos sueltan su rienda en el pensamiento. Dado que el presentismo es inevitable, no debe ser desestimado por completo, sino que debe ser una cuestión sondeada y examinada críticamente. Con dicho foco, el presentismo estratégico en la historiografía debe posibilitar una reflexión sobre los límites de lo que es posible pensar.

  5. Texts of presentation

    Energy Technology Data Exchange (ETDEWEB)

    Magnin, G.; Vidolov, K.; Dufour-Fallot, B.; Dewarrat, Th.; Rose, T.; Favatier, A.; Gazeley, D.; Pujol, T.; Worner, D.; Van de Wel, E.; Revaz, J.M.; Clerfayt, G.; Creedy, A.; Moisan, F.; Geissler, M.; Isbell, P.; Macaluso, M.; Litzka, V.; Gillis, W.; Jarvis, I.; Gorg, M.; Bebie, B.

    2004-07-01

    Implementing a sustainable local energy policy involves a long term reflection on the general interest, energy efficiency, distributed generation and environmental protection. Providing services on a market involves looking for activities that are profitable, if possible in the 'short-term'. The aim of this conference is to analyse the possibility of reconciling these apparently contradictory requirements and how this can be achieved. This conference brings together the best specialists from European municipalities as well as important partners for local authorities (energy agencies, service companies, institutions, etc.) in order to discuss the public-private partnerships concerning the various functions that municipalities may perform in the energy field as consumers and customers, planners and organizers of urban space and rousers as regards inhabitants and economic players of their areas. This document contains the summaries of the following presentations: 1 - Performance contracting: Bulgarian municipalities use private capital for energy efficiency improvement (K. VIDOLOV, Varna (BG)), Contracting experiences in Swiss municipalities: consistent energy policy thanks to the Energy-city label (B. DUFOUR-FALLOT and T. DEWARRAT (CH)), Experience of contracting in the domestic sector (T. ROSE (GB)); 2 - Public procurement: Multicolor electricity (A. FAVATIER (CH)), Tendering for new green electricity capacity (D. GAZELEY (GB)), The Barcelona solar thermal ordinance (T. PUJOL (ES)); 3 - Urban planning and schemes: Influencing energy issues through urban planning (D. WOERNER (DE)), Tendering for the supply of energy infrastructure (E. VAN DE WEL (NL)), Concessions and public utility warranty (J.M. REVAZ (CH)); 4 - Certificate schemes: the market of green certificates in Wallonia region in a liberalized power market (G. CLERFAYT (BE)), The Carbon Neutral{sup R} project: a voluntary certification scheme with opportunity for implementation in other European

  6. Crystallography: past and present

    Science.gov (United States)

    Hodeau, J.-L.; Guinebretiere, R.

    2007-12-01

    structure (chemical order, anisotropy, charge transfer, magnetic order) versus an external parameter like temperature, pressure, magnetic or electric field. Modern crystallography is also extended to the study of very small crystals, powders, ill-ordered or non-crystallized materials. Thus presently, crystallography is concerned with any solid that “scatters” an incident beam. Nevertheless, as quoted by A. Guinier, “the problems facing crystallographers have only changed, ... new ones have appeared which require reflection and imagination, ... and which in turn may still bring much joy to all those who like crystallography” [4]. Such developments open up crystallography to modern materials like artificial ones and nanostructures with low- and/or multi-scaled-periodicities and/or extremely small “crystal size” and to materials of the “real world”, with mixtures of phases and/or amorphous contribution and/or defects, a common characteristic of ancient materials analysed in patrimonial research. In our contribution we will show by selected examples that these improvements were allowed (i) by the use of powerful sources, apparatus and detectors which allow micro-diffraction, in-situ diffraction, spectroscopy, resonant scattering, inelastic scattering, coherent scattering, (ii) by the development of methods like diffraction anomalous fine structure (DAFS), pair distribution function (PDF), simulated annealing, single object reconstruction, (iii) by combination of scattering and spectroscopy and by combination of scattering and microscopy. Such combination of different approaches is very efficient and, as said by H. Curien at the IUCr Bordeaux Congress in 1990, “in crystallography, there is a constant alternation between the crystal space and its associated reciprocal space, ... the alternation between experiment and model building is another feature of crystallography activity ..., the crystallographer relies both on his computer and on his diffractometer

  7. present

    African Journals Online (AJOL)

    as medium of instruction enhances concept formation, especially in science subjects as ... The study, on which this paper is based, was conducted to evaluate Malawi's ... corrected in the wake of information flowing from the evaluation phase (Kaplan .... concerning the background information and suggested activities (Thodi,.

  8. PRESENTATION

    OpenAIRE

    Editors, The

    2017-01-01

    To plan and to implement academic projects remain a systematic challenge for the team and the collaborators of the Electronic Journal. Each study published is an opportunity to strengthen the proposed goals and to add more value to the community involved and interested.

  9. Presentations

    International Nuclear Information System (INIS)

    2007-01-01

    The aim of the Exploratory Workshop is to encourage informal discussion and the exchange of expertise between scientists using random matrix theory in various areas of research ranging from fundamental physics: string theory, gravity, quantum chaos, information theory, complexity theory, combinatorics to applied research: biophysics, econophysics, quantitative finance and telecommunication. Such a discussion would be a first step towards creating a common European environment for researchers using this powerful theory

  10. Tips for Good Electronic Presentations.

    Science.gov (United States)

    Strasser, Dennis

    1996-01-01

    Describes library uses of presentation graphics software and offers tips for creating electronic presentations. Tips include: audience retention; visual aid options; software package options; presentation planning; presentation showing; and use of text, colors, and graphics. Sidebars note common presentation errors and popular presentation…

  11. Punch up your podium presentations.

    Science.gov (United States)

    Wood, Tim J; Hollier, Amelie

    2017-08-01

    The goal of most presentations is to engage audience participants. Effective presenters go beyond content expertise to connect with audiences through various techniques, formats, and presentation styles. Mayer's theory of multimedia learning, neurocognitive factors, and the assertion-evidence approach are essential elements that presenters can use if they have not had formal training in creating presentations. Tips are offered on how to connect with the audience and how to avoid known pitfalls. ©2017 American Association of Nurse Practitioners.

  12. Put Power into Your Presentations: Using Presentation Software Effectively

    Science.gov (United States)

    Safransky, Robert J.; Burmeister, Marsha L.

    2009-01-01

    Microsoft PowerPoint, Apple Keynote, and OpenOffice Impress are relatively common tools in the classroom and in the boardroom these days. What makes presentation software so popular? As the Chinese proverb declares, a picture is worth a thousand words. People like visual presentations. Presentation software can make even a dull subject come to…

  13. Present market for nuclear energy

    International Nuclear Information System (INIS)

    Marzo, M.A.S.

    1987-01-01

    The present market for nuclear energy is present since nuclear production and electric power generation to the utilization of radioisotopes in medicine and biology. Some data about the main world suppliers to this market are shown. (E.G.) [pt

  14. Integral Presentations of Catalan Numbers

    Science.gov (United States)

    Dana-Picard, Thierry

    2010-01-01

    We compute in three different ways the same definite parametric integral. By-products are the derivation of a combinatorial identity and two integral presentations of Catalan numbers. One of them leads to a presentation using the [gamma] function.

  15. An Atypical Presentation on Insulinoma

    Science.gov (United States)

    2017-06-16

    PUBLICATIONS/ PRESENTATIONS 1. TO: CLINICAL RESEARCH 2. FROM: (Author’s Name, Rank, Grade, Office Symbol) 3. GME/GHSE STUDENT: 4. PROTOCOL NUMBER: Kluesner...PROCESSING OF PROFESSIONAL MEDICAL RESEARCH/TECHNICAL PUBLICATIONS/ PRESENTATIONS 1st ENDORSEMENT (59 MDW/SGVU Use Only) TO: Clinical Research Division 24...CAPT JOSEPH KLUESNER FROM: 59 MDW/SGYU SUBJECT: Professional Presentation Approval 1. Your paper, entitled An Atypical Presentation of Insulinoma

  16. Turning Scientific Presentations into Stories

    Science.gov (United States)

    Aruffo, Christopher

    2015-01-01

    To increase students' confidence in giving scientific presentations, students were shown how to present scientific findings as a narrative story. Students who were preparing to give a scientific talk attended a workshop in which they were encouraged to experience the similarities between telling a personal anecdote and presenting scientific data.…

  17. Dressed to Present: Ratings of Classroom Presentations Vary with Attire

    Science.gov (United States)

    Gurung, Regan A. R.; Kempen, Laura; Klemm, Kayla; Senn, Rebecca; Wysocki, Rosie

    2014-01-01

    This study investigates the effects of formality of dress on ratings of classroom presentations. Participants (N = 65, 66% women) from a Midwestern university in the United States rated three female students giving a presentation designed for a health psychology class in one of four outfits: casual, party, business casual, or business formal.…

  18. Isolated Torticollis May Present as an Atypical Presentation of Meningitis

    Directory of Open Access Journals (Sweden)

    Roger Chirurgi

    2012-01-01

    Full Text Available Background. Bacterial meningitis is a life-threatening medical emergency that requires urgent diagnosis and treatment. Diagnosis is infrequently missed if the patient presents with the classic symptoms of fever, headache, rash, nuchal rigidity, or Kernig or Brudzinski sign. However, it may be less obvious in neonates, elderly, or immunocompromised patients. Meningitis which presents as isolated torticollis, without any other signs or symptoms, is exceedingly rare. Objective. To identify an abnormal presentation of meningitis in an adult immunocompromised patient. Case Report. We present a case of an adult diabetic male who presented multiple times to the ED with complaint of isolated torticollis, who ultimately was diagnosed with bacterial meningitis. Conclusion. We propose that in the absence of sufficient explanation for acute painful torticollis in an immunocompromised adult patient, further evaluation, possibly including a lumbar puncture may be warranted.

  19. Discussion paper (presented during meeting)

    International Nuclear Information System (INIS)

    Allemand, R.

    1975-01-01

    A method of particle localization for gas chambers (backgammon game method) is presented. The cathode is geometrically cut in such a way that it is possible to establish a simple relation between the signal amplitude on the different parts of the cathode and the position of the nuclear event. The results obtained in the case of x-ray detectors are presented. The application of this method to the localization of neutrons is presently studied [fr

  20. Unusual presentation of Klinefelter syndrome

    Science.gov (United States)

    Das, Chanchal; Sahana, Pranab Kumar; Sengupta, Nilanjan; Roy, Mukut; Dasgupta, Ranen

    2013-01-01

    Introduction: Klinefelter syndrome usually presents in the puberty and adulthood with its characteristic features. We report a boy who had Klinefelter syndrome with hypospadias and hydrocele. Case Note: Six and half year old boy had complaints of genitourinary problem in the form of hypospadias, small phallus and hydrocele. Karyotyping showed 47,XXY. Conclusion: This case illustrates that Klinefelter syndrome was presented in the infancy with hypospadias and hydrocele which are very uncommon presentation of the disease PMID:24910838

  1. Retinoblastoma: a most unusual presentation

    African Journals Online (AJOL)

    Dr. C. Pedro-Egbe

    Although leukocoria and strabismus are the most common presenting signs of retinoblastoma, a retrospective study by Abramson et al. identified 32 distinct presenting signs of retinoblastoma.6-8. A few other studies have also noted some distinct characteristics exhibited by children whose conditions were diagnosed in the ...

  2. Present status of processing method

    Energy Technology Data Exchange (ETDEWEB)

    Kosako, Kazuaki [Sumitomo Atomic Energy Industries Ltd., Tokyo (Japan)

    1998-11-01

    Present status of processing method for a high-energy nuclear data file was examined. The NJOY94 code is the only one available to the processing. In Japan, present processing used NJOY94 is orienting toward the production of traditional cross section library, because a high-energy transport code using a high-energy cross section library is indistinct. (author)

  3. Uncommon presentation of neuro- cysticercosis

    African Journals Online (AJOL)

    Uncommon presentation of neuro- cysticercosis. Neurocysticercosis (NCC) is a common parasitic infection of the central nervous system caused by the pork tapeworm Taenia solium. The clinical presentation of NCC in children includes generalised and partial seizures with or without features of raised intracranial pressure.

  4. Presentation Skills Workshops for Nurses.

    Science.gov (United States)

    Kinn, S.; Kenyon, M.

    2002-01-01

    Workshops were held to prepare nurses (n=87) to present results of professional activities. One year after the course, 20 had made oral and 30 written presentations. The workshops increased their confidence and were considered practical, informal, and nonthreatening. (Contains 31 references.) (SK)

  5. Castleman's disease, an unusual presentation

    Directory of Open Access Journals (Sweden)

    Shital Munde

    2015-01-01

    The present report describes a 30-year-old serving male who presented with pain abdomen and on and off episodes ofmelena, found to have peripancreatic lymphadenopathy on CECT abdomen. Ultrasound guided FN AC was done in private hospital revealed reactive lymphadenopathy. Excision biopsy was done in our institute and a diagnosis of Castleman's disease was made.

  6. Multiphasic presentation of Rasmussen's encephalitis

    NARCIS (Netherlands)

    Avbersek, A.; Miserocchi, A.; McEvoy, A.W.; Patel, A.V.; Aronica, E.; Blumcke, I.; Jacques, T.S.; Acheson, J.; Thom, M.; Sisodiya, S.M.

    2015-01-01

    Rasmussen's encephalitis is a rare, chronic inflammatory disorder of unknown cause, characterised by drug-resistant focal epilepsy that may rarely present in adolescence or adulthood. We present a case of Rasmussen's encephalitis with prominent recurrent fluctuation in symptoms and well-documented

  7. Creating an effective poster presentation.

    Science.gov (United States)

    Taggart, H M; Arslanian, C

    2000-01-01

    One way to build knowledge in nursing is to share research findings or clinical program outcomes. The dissemination of these findings is often a difficult final step in a project that has taken months or years to complete. One method of sharing findings in a relaxed and informal setting is a poster presentation. This method is an effective form for presenting findings using an interactive approach. The milieu of a poster presentation enables the presenters to interact and dialogue with colleagues. Guidelines for size and format require that the poster is clear and informative. Application of design helps to create visually appealing posters. This article summarizes elements of designing and conducting a poster presentation.

  8. Atypical presentations of Wolframs syndrome

    Directory of Open Access Journals (Sweden)

    S Saran

    2012-01-01

    Full Text Available Background: Wolfram syndrome is a rare hereditary or sporadic neurodegenerative disorder also known as DIDMOAD. The classically described presentation is of insulin-dependent diabetes, followed by optic atrophy, central diabetes insipidus, and sensory neural deafness. Also included are less well-described presentations of Wolframs syndrome. We here present three cases of atypical presentation of this syndrome. Case 1: A 15-year-old boy with insulin-dependent diabetes was presented for evaluation of depressive symptoms associated with suicidal tendency. Neuropsychiatric manifestations are described with Wolframs syndrome, and wolframin gene, in recessive inheritance, is associated with psychiatric illnesses without other manifestations of Wolframs syndrome. Case 2: A 17-year-old diabetic boy on insulin with good control of blood sugar presented for evaluation of delayed puberty. Central hypogonadism and other anterior pituitary hormone dysfunctions are the less publicized hormone dysfunctions in Wolframs syndrome. Case 3: A 23-year-old female who was on insulin for diabetes for the past 14 years, got admitted for evaluation of sudden loss of vision. This patient had developed a vitreous hemorrhage and, on evaluation, was found to have optic atrophy, sensory neural hearing loss, and diabetes insipidus, and presented differently from the gradual loss of vision described in Wolframs syndrome. Conclusion: Wolframs syndrome being a multisystem degenerative disorder can have myriad other manifestations than the classically described features. Neuropsychiatric manifestations, depression with suicidal risk, central hypogonadism, and secondary adrenal insufficiency are among the less well-described manifestations of this syndrome.

  9. An unusual presentation of sporotrichosis

    Directory of Open Access Journals (Sweden)

    S S Vaishampayan

    2013-01-01

    Full Text Available Sporotrichosis can mimic a few diseases. Sporotrichosis presenting as a hypoaesthetic plaque or plaque suggestive of leprosy has not been mentioned in literature. Herein we present a case of a 26 years old man who presented with hypoesthetic plaque on left ankle and was clinically diagnosed to have borderline tuberculoid leprosy who later developed few nodules in linear fashion on left leg, which ruptured to form non healing ulcers. Culture from ulcer grew Sporothrix schenckii. Patient was successfully treated with oral potassium iodide.

  10. Rhabdomyosarcoma presenting as acute leukemia.

    Science.gov (United States)

    Morandi, S; Manna, A; Sabattini, E; Porcellini, A

    1996-08-01

    We describe a case of a very unusual presentation of rhabdomyosarcoma. An 18-year-old woman presented with symptoms and signs compatible with acute leukemia. The bone marrow picture showed diffuse involvement sustained by undifferentiated blasts that turned out to be of striated muscle origin by immunochemistry. While it is well known that rhabdomyosarcoma may metastasize to the bone marrow, extensive marrow involvement with leukemic spread as a unique clinical manifestation is extremely rare. Our observation further confirms the need to consider rhabdomyosarcoma among the possible differential diagnoses in patients who present with a leukemic picture and atypical blasts lacking all hematopoietic markers.

  11. Clinical presentation of thyroid cancer

    International Nuclear Information System (INIS)

    Samuel, A.M.; Shah, D.H.

    1999-01-01

    The clinical manifestation of thyroid cancer (TC) as seen at the Nuclear Medicine Department, where the patients investigated prior to diagnosis of disease are clinically suspected to harbor malignancy and mostly referred for scintigraphic investigations are presented

  12. Hyperthyroidism: an unusual case presentation.

    Science.gov (United States)

    Scripture, D L

    1998-02-01

    Hyperthyroidism is the most common disorder of the thyroid. Patients typically present with complaints consistent with a hypermetabolic state, including nervousness, weight loss, heat intolerance, palpitations, irritability, and tremor. This case report reviews a 34-year-old woman who presented with unilateral upper extremity weakness, weight gain, and an episode of atrial fibrillation, the latter coinciding with a 36-hour lack of sleep and excess alcohol and caffeine intake. Although an extensive neurologic evaluation failed to identify any abnormality, the patient's laboratory analysis revealed elevations in thyroxine (T4) and triiodothyronine (T3) levels with unsuppressed thyroid-stimulating hormone levels. Subsequent treatment with the antithyroid drug methimazole (Tapazole) provided complete relief of symptoms. This case report illustrates how health care providers can be diverted to pursue a neurologic etiology when muscle weakness presents as a unilateral symptom. Plausible alternative causes for muscle weakness and other symptoms are presented.

  13. Thyrotoxic crisis presenting with jaundice.

    Science.gov (United States)

    Wickramasinghe, R D S S; Luke, W A N V; Sebastiampillai, B S; Gunathilake, M P M L; Premaratna, R

    2016-06-23

    Thyrotoxic crisis is a medical emergency requiring early diagnosis and urgent management, which can be challenging due to its diverse clinical presentations. While common presentations include fever, sweating, palpitations, tremors and confusion, presence of jaundice is rare. We report a 35-year-old male who presented with jaundice due to cholestasis along with other features of thyrotoxic crisis due to Graves' disease. He had a good clinical recovery with resolution of cholestasis following treatment for thyrotoxic crisis. Jaundice can be a rare manifestation of thyrotoxic crisis, and should be considered in the differential diagnosis when other clinical features of thyrotoxic crisis are present. However secondary causes of jaundice should be looked into and excluded.

  14. Presentation at FTP: A review

    International Nuclear Information System (INIS)

    Sheffield, J.

    1987-01-01

    This series of vugraphs presents some information concerning ORNL's involvement in the fusion energy research program. Recent and future experiments are named, and a rough estimate of funding is given

  15. Extradural lipomatosis presenting with paraplegia.

    Directory of Open Access Journals (Sweden)

    Deogaonkar M

    1995-07-01

    Full Text Available An unusual case with spinal extradural lipomatosis in a non-obese and otherwise healthy man is reported. The patient presented with a history of weakness of legs which progressed to paraplegia over a 40 day period.

  16. A centralized audio presentation manager

    Energy Technology Data Exchange (ETDEWEB)

    Papp, A.L. III; Blattner, M.M.

    1994-05-16

    The centralized audio presentation manager addresses the problems which occur when multiple programs running simultaneously attempt to use the audio output of a computer system. Time dependence of sound means that certain auditory messages must be scheduled simultaneously, which can lead to perceptual problems due to psychoacoustic phenomena. Furthermore, the combination of speech and nonspeech audio is examined; each presents its own problems of perceptibility in an acoustic environment composed of multiple auditory streams. The centralized audio presentation manager receives abstract parameterized message requests from the currently running programs, and attempts to create and present a sonic representation in the most perceptible manner through the use of a theoretically and empirically designed rule set.

  17. Hypertension presenting early in pregnancy

    OpenAIRE

    Melvin, Audrey; Kinsley, Brendan

    2015-01-01

    Key Clinical Message Paraganglioma in pregnancy is an exceedingly rare and potentially life?threatening diagnosis. It is important that the clinicians consider secondary causes when women present with hypertension in early pregnancy.

  18. HYDROGEOLOGIC CASE STUDIES (DENVER PRESENTATION)

    Science.gov (United States)

    Hydrogeology is the foundation of subsurface site characterization for evaluations of monitored natural attenuation (MNA). Three case studies are presented. Examples of the potentially detrimental effects of drilling additives on ground-water samples from monitoring wells are d...

  19. QCD machines - present and future

    International Nuclear Information System (INIS)

    Christ, N.H.

    1991-01-01

    The present status of the currently working and nearly working dedicated QCD machines is reviewed and proposals for future machines are discussed with particular emphasis on the QCD Teraflop Project in the US. (orig.)

  20. Unusual presentation of cutaneous leiomyoma

    Directory of Open Access Journals (Sweden)

    Sapnashree Bhaskar

    2014-01-01

    Full Text Available Herein, we report a case of leiomyoma cutis because of its rarity and unusual presentation. The case presented with a solitary leiomyoma lesion which was painless. However, the adjacent normal appearing area was tender. A biopsy of the lesion as well as of a portion of the adjacent normal appearing area was taken, which confirmed the diagnosis of cutaneous leiomyoma. This may suggest the dormant nature of the disease which has not yet become apparent.

  1. ECRIS 2010 - Proceedings and Presentations

    International Nuclear Information System (INIS)

    2012-01-01

    The workshop focussed on the latest developments in performance, modeling and applications of ECR ion sources along with the associated physics and technologies, including plasma physics, beam optics, beam diagnostics, magnet technology.... The presentations were organized around 8 topics: 1) status of ECRIS operation, 2) new developments, 3) ion beams and low energy beam transport (LEBT), 4) pulsed operation, 5) applications, and 6) plasma and beam investigations. Most papers are accompanied with the slides of their presentation

  2. AN UNUSUAL PRESENTATION OF DIC

    Directory of Open Access Journals (Sweden)

    Manish

    2015-10-01

    Full Text Available Placenta Praevia is a common obstetric problem which presents with antepartum H aemorrhage and is treated by the obstetrician. It is very rare to see a patient with placenta praevia treated in medical ward for post - partum H aemorrhage due to disseminated intravascular coagulation defect with the added complication of acute kidney injury, P ost - partum H ypertension and PRES (posterior reversible encephalopathy syndrome, hence the presentation

  3. Present and future superconducting cyclotrons

    International Nuclear Information System (INIS)

    Nolen, J.A. Jr.

    1987-01-01

    This paper begins with a brief review of the status of present superconducting (SC) cyclotron projects, including the two which are currently operating and the six which are under construction. The next section summarizes the main features shared by five of these machines, while the third section presents recent developments and new concepts introduced in the other three ''second generation'' SC cyclotrons. Projects in early stages of development are discussed in the fourth section

  4. Peripartum Cardiomyopathy Presenting as Bradycardia

    OpenAIRE

    Codsi, Elisabeth; Rose, Carl H.; Tweet, Marysia S.; Hayes, Sharonne N.; Best, Patricia J. M.; Blauwet, Lori A.

    2017-01-01

    Peripartum cardiomyopathy (PPCM) is a disease that typically affects young otherwise healthy women. As PPCM is associated with significant mortality, timely diagnosis is necessary to ensure appropriate care. To our knowledge, this represents the first reported case of PPCM presenting as symptomatic bradycardia. We describe the patient’s clinical presentation and relevant findings and review the potential etiology and ramifications of bradycardia in patients with PPCM.

  5. Presentation of the ASTRAL software; Presentation du logiciel ASTRAL

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    2011-07-01

    This report presents the ASTRAL software (ASTRAL means technical assistance in post-accidental radioprotection) which is aimed to be used as an aid-to-decision tool in the case of an important release of radionuclides in the environment by assessing radionuclide concentration in different environments and food products, determining the potential exposure to irradiation of concerned populations, foreseeing the evolution of the situation, and proposing different scenarios for the management of contaminated areas. The report describes the software general operation, presents the calculation module (main functionalities, concentration index calculation, dose calculation or radiological impact calculation, how countermeasures are taken into account), the data bases (contextual data, data for radio-ecological calculations and for radiological calculations), the software ergonomics (general principles, result selection and display, result printing and input data). Its briefly evokes the development quality assurance, and describes the software implementation architecture

  6. Dexmedetomidine overdosage: An unusual presentation

    Directory of Open Access Journals (Sweden)

    Soumya S Nath

    2013-01-01

    Full Text Available We present a case of dexmedetomidine toxicity in a 3-year-old child. The case report describes the features and outlines the treatment strategy adopted. The child presented with bradypnoea, bradycardia, hypotension, deep hypnosis and miosis. He was successfully managed with oxygen, saline boluses and adrenaline infusion. He became haemodynamically stable with adrenaline infusion. He started responding to painful stimuli in 3 h and became oriented in 7 h. Dexmedetomidine, a selective α2 adrenoceptor agonist, is claimed to have a wide safety margin. This case report highlights the fact that dexmedetomidine administered in a toxic dose may be life-threatening may present with miosis and adrenaline infusion may be a useful supportive treatment.

  7. Neonatal hemophilia: a rare presentation

    Directory of Open Access Journals (Sweden)

    Nuno Ferreira

    2015-12-01

    Full Text Available Hemophilia A is a X-linked hereditary condition that lead to decreased factor VIII activity, occurs mainly in males. Decreased factor VIII activity leads to increased risk of bleeding events. During neonatal period, diagnosis is made after post-partum bleeding complication or unexpected bleeding after medical procedures. Subgaleal hemorrhage during neonatal period is a rare, severe extracranial bleeding with high mortality and usually related to traumatic labor or coagulation disorders. Subgaleal hemorrhage complications result from massive bleeding. We present a neonate with unremarkable family history and uneventful pregnancy with a vaginal delivery with no instrumentation, presenting with severe subgaleal bleeding at 52 hours of life. Aggressive support measures were implemented and bleeding managed. The unexpected bleeding lead to a coagulation study and the diagnosis of severe hemophilia A. There were no known sequelae. This case shows a rare hemophilia presentation reflecting the importance of coagulation studies when faced with unexplained severe bleeding.

  8. Graphical presentation of participants' results

    International Nuclear Information System (INIS)

    Anon.

    1991-01-01

    The results obtained by 19 participating dosimetry systems are presented in this section. Section 3 of the preceding report, ''Evaluation of the Results of the First IAEA Coordinated Research Program on Intercomparison for Individual Monitoring'', explains the format and conventions used for the graphical presentation. The system number appearing in the upper left hand corner of each page can be used to correlate the dosimeter design information found in Table 2 of the preceding report with the results. However, care should be used in drawing inferences from this correlation, since system performance depends on a number of factors other than detector selection and design characteristics. (orig.)

  9. Technical presentation: Next Generation Oscilloscopes

    CERN Multimedia

    PH Department

    2011-01-01

      Rohde & Schwarz "Next Generation Oscilloscopes" - Introduction and Presentation Agenda: Wednesday 23 March  -  09:30 to 11:30 (open end) Bldg. 13-2-005 Language: English 09.30 Presentation "Next Generation Oscilloscopes" from Rohde & Schwarz RTO / RTM in theory and practice Gerard Walker 10.15 Technical design details from R&D Dr. Markus Freidhof 10.45 Scope and Probe Roadmap (confidential) Guido Schulze 11.00 Open Discussion Feedback, first impression, wishes, needs and requirements from CERN All 11.30 Expert Talks, Hands on All Mr. Dr. Markus Freidhof, Head of R&D Oscilloscopes, Rohde & Schwarz, Germany; Mr. Guido Schulze, ...

  10. DOE Collegiate Wind Competition (Presentation)

    Energy Technology Data Exchange (ETDEWEB)

    Jones, J.

    2014-02-01

    This presentation for the January Stakeholder Engagement and Outreach webinar outlines the expanded need for workers in the wind industry and provides an overview of the DOE Wind Competition (to be held in May 2014) and the guiding principles of the competition.

  11. Press book presentation progress report

    International Nuclear Information System (INIS)

    2001-07-01

    This document presents four french sites of the ANDRA concerning the radioactive waste disposal. The concerned site are the underground research laboratory of Meuse/Haute-Marne, the Aube Center, the TFA Center and the Manche Center. For each sites, data and information on the geophysics, the capacity, the management or the research programs are analysed. (A.L.B.)

  12. Rose breeding: past, present, prospects

    NARCIS (Netherlands)

    Vries, de D.P.; Dubois, L.A.M.

    1996-01-01

    In this review the PAST, PRESENT and PROSPECT will be considered as three separate periods in the history of the breeding and development of rose cultivars. The recurring theme is the genetic variation. This theme was chosen because there is justified doubt as to sufficient genetic variation

  13. Understanding Algorithms in Different Presentations

    Science.gov (United States)

    Csernoch, Mária; Biró, Piroska; Abari, Kálmán; Máth, János

    2015-01-01

    Within the framework of the Testing Algorithmic and Application Skills project we tested first year students of Informatics at the beginning of their tertiary education. We were focusing on the students' level of understanding in different programming environments. In the present paper we provide the results from the University of Debrecen, the…

  14. Experiments in Multimodal Information Presentation

    NARCIS (Netherlands)

    van Hooijdonk, Charlotte; Bosma, W.E.; Krahmer, Emiel; Maes, Alfons; Theune, Mariet; van den Bosch, Antal; Bouma, Gosse

    In this chapter we describe three experiments investigating multimodal information presentation in the context of a medical QA system. In Experiment 1, we wanted to know how non-experts design (multimodal) answers to medical questions, distinguishing between what questions and how questions. In

  15. Clinical Presentations of Acute Leukemia

    International Nuclear Information System (INIS)

    Shahab, F.; Raziq, F.

    2014-01-01

    Objective: To document the clinical presentation and epidemiology of various types of acute leukemia with their respective referral source at a tertiary level centre in Peshawar. Study Design: An observational study. Place and Duration of Study: Department of Pathology, Hayatabad Medical Complex (HMC), Peshawar, from January 2011 to May 2012. Methodology: A total of 618 bone marrow biopsy reports were reviewed. All biopsy reports labeled as acute leukemia were reviewed for age, gender, address, referring unit, diagnosis on bone marrow examination, presenting complaints, duration of illness and findings of clinical examination. Results: Ninety-two patients were diagnosed as suffering from acute leukemias (15%). ALL was most prevalent (46%), followed by AML (38%) and undifferentiated acute leukemia (16%). Males were affected more compared to females (60% vs. 40%). ALL and AML were predominant in pediatric (64%) and adults (77%) patients respectively. Patients from Afghanistan accounted for 33% of all cases followed by Peshawar (14%). Fever (77%), pallor (33%) and bleeding disorders (23%) were the main presenting complaints. Enlargement of liver, spleen and lymph nodes together was associated with ALL compared with AML (p = 0.004). Conclusion: ALL-L1 and AML-M4 were the most common sub-types. Fever, pallor and bleeding disorders were the main presenting complaints. Enlargement of liver, spleen and lymph nodes was more frequently associated with ALL compared to AML. (author)

  16. Reninoma presenting as cardiac syncope

    Science.gov (United States)

    Tak, Shahid I; Wani, Mohd Lateef; Khan, Khursheed A; Alai, Mohd Sultan; Shera, Altaf Hussain; Ahangar, Abdul G; Khan, Yasir Bashir; Nayeem-ul-Hassan; Irshad, Ifat

    2011-01-01

    Reninoma, a renin-secreting tumor of the juxta-glomerular cells of the kidney, is a rare but surgically treatable cause of secondary hypertension in children. We report a case of reninoma presenting as cardiac syncope with long QTc on electrocardiogram due to hypokalemia. PMID:21677812

  17. Unusual Presentation of Maydl's Hernia

    African Journals Online (AJOL)

    Nikhil NBA, Natarajan K, Mohanty A, et al. An. Unusual Case of Maydl's Hernia. Int J Cur Res Rev. 2013;5(6):22-5. 11. Ganesaratnam M. Maydl's hernia: Report of a Series of Seven Cases and Review of Literature. Brit J Surg. 1985;72:737-8. 12. Weledji EP, Mokake M, Ngowe MN. A Rare. Presentation of Maydl's Hernia.

  18. Secondary syphilis presenting as vertigo

    International Nuclear Information System (INIS)

    Bari, A.U.; Mehmood, T.; Khan, B.; Malik, N.; Malik, K.Z.; Sukhera, A.M.

    2006-01-01

    Syphilis is a chronic, systemic and sexually transmitted infectious disease affecting most of the organs in the body. A young African man presented with vertigo, unsteadiness of gait and a skin rash suggestive of secondary syphilis. Diagnosis was confirmed on serology and was treated with two shots of long-acting penicillin, following which his symptoms settled. (author)

  19. Energy consumption: Past, present, future

    Science.gov (United States)

    1973-01-01

    The energy consumption history of the United States and the changes which could occur in consumption characteristics in the next 50 years are presented. The various sources of energy are analyzed to show the limitations involved in development and utilization as a function of time available. Several scenarios were prepared to show the consumption and supply of energy under varying conditions.

  20. Learning through Dramatic Story Presentation

    Science.gov (United States)

    Tindall, Evie

    2012-01-01

    The use of story with dramatic presentation approaches produces an engaging and powerful instructional choice for today's adult ESL educators. Two engaging and timed-tested approaches are Reader's Theater and Tableau Vivant. Both provide English language learners with content tailored to their abilities in addition to numerable opportunities to…

  1. Police, managerialization and presentational strategies

    NARCIS (Netherlands)

    Terpstra, J.; Trommel, W.A.

    2009-01-01

    Purpose - The managerialization of the police may be seen as an effort to restore the legitimacy of the police. This paper aims to show that the managerialist strategy presently occurring within police forces creates new pitfalls and unintended consequences. Design/methodology/approach - The paper

  2. Viewfoils for environmental capabilities presentation

    International Nuclear Information System (INIS)

    1992-01-01

    This document contains information about the environmental applications of aerial surveys. It discusses the accuracy, dependability, history, advantages, and sensitivity of aerial surveys. Also included, are a brief overview of in-situ gamma spectroscopy and samples of aerial surveys. This document contains entirely of an outline and viewfoils for the presentation

  3. Present Day Philosophies of Education

    Science.gov (United States)

    Ediger, Marlow

    2006-01-01

    Presently, there are competing philosophies of education which need comparison. Two philosophies will be compared which are at opposite ends of the continuum. They are distinctly different. And yet, both schools of thought have their disciples. Each of the two will be discussed in terms of its essential features and then there will be selected…

  4. MAMMARY TUBERCULOSIS PRESENTING AS CARCINOMA

    African Journals Online (AJOL)

    She was otherwise well wizh no symptoms referable to other systems. On examination .... cardiac failure. The other ... but chronic oedema of the right arm of moderate degree was present. .... a tuberculous ulcer on the dorsum of the hand, followed ... venous system may also lead to haematogenous dissemination, producing ...

  5. The Present State of Behaviorism

    Science.gov (United States)

    Mowrer, O. Hobart

    1976-01-01

    Assesses the present status of behaviorism by dividing this study into an evaluation of Behaviorism 1, based explicitly on Pavlov's conception of conditioned-response learning, and Behaviorism 11, single-handedly launched and almost completely dominanted by Professor B. F. Skinner. (Author/RK)

  6. Petroleum - its present and future

    Digital Repository Service at National Institute of Oceanography (India)

    SenGupta, R.; Qasim, S.Z.

    World's "published proved" reserve of oil by the end of 1984 was known to be of the order of 95,000 million tonnes. Of this, 57% lies buried in the middle eastern countries. At the present rate of growth in consumption and production of oil...

  7. Reactor enclosure. BRC meeting presentation

    International Nuclear Information System (INIS)

    Fisch, J.W.

    1975-01-01

    The latest status of key components of the Reactor Enclosure System of the Clinch River Breeder Reactor Plant is described. Areas where there have been notable design changes or significant design detail maturity in the six months since the last BRC presentation are highlighted. (auth)

  8. Slide Composition for Electronic Presentations

    Science.gov (United States)

    Larson, Ronald B.

    2004-01-01

    Instructors who use computer-generated graphics in their lectures have many options to consider when developing their presentations. Experts give different advice on which typefaces, background and letter colors, and background imagery improve communications. This study attempted to resolve these controversies by examining how short-term recall of…

  9. Hypervideos and interactive multimedia presentations

    NARCIS (Netherlands)

    B. Meixner (Britta)

    2017-01-01

    textabstractHypervideos and interactive multimedia presentations allow the creation of fully interactive and enriched video. It is possible to organize video scenes in a nonlinear way. Additional information can be added to the video ranging from short descriptions to images and more videos.

  10. Unusual presentation of gallbladder perforation

    Directory of Open Access Journals (Sweden)

    G. Jayasinghe

    2016-01-01

    Conclusions: Percutaneous abscesses arising from the gallbladder are a rare but potentially serious consequence of acute cholecystitis, and may present in a wide variety of locations. Therefore it is imperative to conduct a full body inspection in the septic patient, even when a source has been identified.

  11. Neutrino astronomy: Present and future

    Indian Academy of Sciences (India)

    Abstract. I briefly review the present and future status of the burgeoning field of neu- ... near future build upon previous results. ... novel radio and acoustic techniques utilized by, for example, experiments such as. GLUE ... Left: Schematic of νµ-induced muon track in detector. Right: .... an eye toward the galactic center).

  12. BVDV: past, present, and future

    Science.gov (United States)

    The term bovine viral diarrhea (BVD) has come to refer to a collection of diverse clinical presentations that include respiratory, enteric and reproductive diseases accompanied by immunosuppression. BVD may be caused by one of three different species of bovine pestivirus, bovine viral diarrhea viru...

  13. Clustering semantics for hypermedia presentation

    NARCIS (Netherlands)

    Alberink, M.J.; Rutledge, L.W.; Hardman, H.L.; Veenstra, M.J.A.

    2004-01-01

    Semantic annotations of media repositories make relationships among the stored media and relevant concepts explicit. However, these relationships and the media they join are not directly presentable as hypermedia. Previous work shows how clustering over the annotations in the repositories can

  14. Summary of presentations and discussions

    International Nuclear Information System (INIS)

    Takeuchi, Mitsuo

    2008-01-01

    In December 2007, the Forum on Stakeholder Confidence discussed its theme entitled 'Link between research, development and demonstration (RD and D) and stakeholder confidence'. It was remarked that regulators need a technical demonstration to aid in evaluating the safety case. Local stakeholders appreciate the opportunity to visualise technological arrangements. In both cases, demonstration adds to confidence in the feasibility of solutions. Some believe there is an important role for analogues in communication with stakeholders, if handled with integrity. To explore and benchmark current practices, it was decided to hold a topical session at the 9. regular meeting of the FSC on 4 June 2008 regarding the use of analogues for confidence building. The session opened with an introductory presentation by the session rapporteur. This incorporated input provided for the purpose by FSC members in cooperation with their country's representative to the NEA RWMC 'Integration Group on the Safety Case'. Three speakers then presented the various uses of analogues by implementers, regulators and scientists to build their own confidence; a fourth speaker dealt with the experience of using natural analogues in public information. The presentations addressed the use of analogues in the field of geological disposal of high-level waste (HLW) and long-lived intermediate level (ILW-LL) radioactive waste. Then the FSC participants split into two working groups for discussion. The outcome of these discussions was reported in plenary on 6 June 2008 and it was agreed to publish proceedings of the session. The present summary, prepared by the session rapporteur with input from the NEA Secretariat, captures the main points heard in the course of the event. It combines data from the formal presentations and remarks made in discussion. The latter represent viewpoints expressed by a group whose primary focus is not natural analogues but rather stakeholder interests. The summary and viewpoints

  15. Pulmonary hyalinizing granuloma presenting with dysphagia: a rare presentation.

    Science.gov (United States)

    Khan, Fazal; Hamid, Arsalan; Fatima, Benish; Hashmi, Shiraz; Fatimi, Saulat

    2017-01-01

    A 25-year-old man presented with a 2-month history of dysphagia and past history of pulmonary and intestinal tuberculosis. A barium swallow showed a point of constriction 42 mm above the gastroesophageal junction. Computed tomography revealed large opacities in bilateral lung fields, encroaching more on the esophagus. The lesion progressively compressed the esophagus as it moved inferiorly. A right posterolateral thoracotomy was performed for sub-anatomical resection of the mass. A biopsy revealed homogenous whirling hyalinized collagen fibers, highly suggestive of pulmonary hyalinizing granuloma, with no evidence of malignancy. Pulmonary hyalinizing granuloma should be considered in the differential diagnosis of longstanding dysphagia.

  16. An unusual presentation of presentation of a branchial cleft cyst.

    Science.gov (United States)

    Vemula, Rahul; Greco, Gregory

    2012-05-01

    Branchial cleft cysts are congenital anomalies that arise from the aberrant embryological development of the branchial apparatus. The location of a branchial cleft cyst is determined by which branchial cleft failed to obliterate during embryological development, with the second branchial cleft cyst being the most commonly recognized lesion. Although the most common location for branchial cleft cysts is between the external auditory canal and the level of the clavicle, the literature does describe unusual locations. We present a case a 15-year-old boy who had an enlarging lesion on his back that had been present since birth. A presumptive radiologic diagnosis of lymphangioma circumscriptum was made. Upon excision of the lesion and pathologic examination, it was determined to be a branchial cleft cyst. The patient had an uneventful postoperative course, and no recurrence was noted after a 2-year follow-up. Our clinical report demonstrates a lesion on the posterior thorax that proved to be a branchial cleft cyst and should always be part of the differential diagnosis for soft tissue masses of the thorax.

  17. Presentation of the ASTRAL software

    International Nuclear Information System (INIS)

    2011-01-01

    This report presents the ASTRAL software (ASTRAL means technical assistance in post-accidental radioprotection) which is aimed to be used as an aid-to-decision tool in the case of an important release of radionuclides in the environment by assessing radionuclide concentration in different environments and food products, determining the potential exposure to irradiation of concerned populations, foreseeing the evolution of the situation, and proposing different scenarios for the management of contaminated areas. The report describes the software general operation, presents the calculation module (main functionalities, concentration index calculation, dose calculation or radiological impact calculation, how countermeasures are taken into account), the data bases (contextual data, data for radio-ecological calculations and for radiological calculations), the software ergonomics (general principles, result selection and display, result printing and input data). Its briefly evokes the development quality assurance, and describes the software implementation architecture

  18. Presentation of economic evaluation results.

    Science.gov (United States)

    Chaikledkaew, Usa

    2014-05-01

    The first HTA guidelines for Thailand included a chapter outlining a set of guidelines on how best to report the findings of health economic evaluations, based on a review of best practice and existing guidelines on the presentation of economic evaluation results from around the world. In this second edition of HTA guidelines for Thailand, the recommendations build on the first edition by using a case study to illustrate how the guidelines can be applied in a real research context. The guidelines propose that all reporting include ten key elements: defining the scope of the study, selection of comparator(s), defining the type of economic evaluation, measurement of costs, measurement of clinical effects, handling time in economic evaluation studies, handling uncertainty and sensitivity analysis, presentation of the results, discussion of the results, and disclosure of funding and authors conflict of interest.

  19. RTNS-II: present status

    International Nuclear Information System (INIS)

    Heikkinen, D.W.; Logan, C.M.

    1980-10-01

    The present status of the RTNS-II facility is described and typical operating parameters are given. A brief discussion is given of the methods used in production of the TiT 2 targets as well as their performance and tritium handling at RTNS-II. The various types of non-interactive beam diagnostics presently in use at the neutron sources are outlined. The on-line computer system which provides a time history of an irradiation and records target performance is described. Examples are listed of several representative experimental programs which have been carried out thus far at RTNS-II. These include both active and passive experiments. Finally, several of the major improvements to the facility made since the beginning of the experimental program are given

  20. Presentation of TVO's visitor's centre

    International Nuclear Information System (INIS)

    Aemmaelae, V.M.

    1993-01-01

    There are four nuclear power plant units in Finland, two of which are PWR's owned by Imatran Voima Oy. The two BWR units are located at Olkiluoto and owned by Teollisuuden Voima Oy. This presentation tells about TVO's concept of informing the visitors at Olkiluoto. At the site there are located, in addition to the two nuclear power plant units, the intermediate storage for spent fuel, the repository for low and medium-active waste as well as the training centre. At the Olkiluoto Visitor's Centre all the activities of the company are presented using varied audio-visual aids. The centre has several exhibits and there are also different installations to show how the plant works. (author)

  1. Amebiasis Presenting as Pleuropulmonary Disease

    OpenAIRE

    Kubitschek, Kenneth R.; Peters, Jay; Nickeson, David; Musher, Daniel M.

    1985-01-01

    Seven patients with amebic liver abscess presenting as pleuropulmonary disease were admitted to hospital initially because of pulmonary symptoms and were found to have amebic liver disease. Three categories of pleuropulmonary involvement included reactive inflammation of the pleura or lung, rupture of a hepatic abscess into the pleural space and rupture of a hepatic abscess into the bronchial airways. The preferred medical treatment is with metronidazole, but rupture of hepatic amebic abscess...

  2. Computer training, present and future.

    Science.gov (United States)

    Smith, G. A.

    1972-01-01

    The products of educational firms today lead toward a multimedia approach to the education and training of commercial programmers and systems analysts. Computer-assisted instruction or CAI is a relatively new medium to augment the other media. The government use of computers is discussed together with the importance of computer pretests. Pretests can aid in determining a person's ability to absorb a particular instructional level. The material presented in a number of computer courses is listed.

  3. Sarcoidosis presenting as penile mass

    Directory of Open Access Journals (Sweden)

    Hüseyin Semiz

    2017-01-01

    Full Text Available Sarcoidosis is an inflammatory disease with unknown cause characterized by noncaseating granuloma formations. It may present with bilateral hilar lymphadenopathy, skin lesions, eye, and musculoskeletal system involvement. Rare involvement of the genital organs (prostate, testis, epididymis has also been reported. However, penile involvement is observed quite rare. In this paper, we report a patient with penile mass who was diagnosed with sarcoidosis on the basis of the laboratory, radiological, and pathological investigations.

  4. Carcinoma caecum - a rare presentation

    International Nuclear Information System (INIS)

    Ali, T.; Mudasser, S.; Mudsser, S.; Sarwar, M.

    2010-01-01

    Caecal and right sided colonic cancers mostly present with fatigue, weakness and iron deficiency anemia. Such tumours rarely perforate posteriorly and involve the retroperitoneum. We report a case of an old Omani lady who came with insidious sign and symptoms of perforated caecum leading to retroperitoneal collection and necrotizing fasciitis of abdominal wall due to carcinoma of caecum. She underwent surgery but despite active intervention, she died because of septicemic shock. (author)

  5. Water plants past and present

    DEFF Research Database (Denmark)

    Riis, Tenna

    2006-01-01

    How has the condition of the environment developed during the last 100 years? This is an important question because the conditions of the past infl uence those of the present, and knowledge about this development makes it possible to evaluate the causes of changes in species richness, species com...... composition and environmental quality. Here we compare old and contemporary studies of the Danish stream fl ora....

  6. Burning mouth syndrome: Present perspective

    OpenAIRE

    Ramesh Parajuli

    2015-01-01

    Introduction: Burning mouth syndrome is characterized by chronic oral pain or burning sensation affecting the oral mucosa in the absence of obvious visible mucosal lesions. Patient presenting with the burning mouth sensation or pain is frequently encountered in clinical practice which poses a challenge to the treating clinician. Its exact etiology remains unknown which probably has multifactorial origin. It often affects middle or old age women and it may be accompanied by xerostomia and alte...

  7. Present status of CP violation

    International Nuclear Information System (INIS)

    Ng, J.N.

    1989-06-01

    A review of the status of CP violation in kaons is given. Status of our knowledge of quark mixing angles in the standard six quark model is presented. The role Β d o - Βd o transition plays in this study is examined. A comparison of the estimates of CP violation effects from models beyond the standard one is given. Other experiments that have the capability of testing different CP violation models are also discussed. (Author) 35 refs., 6 figs., tab

  8. Nocardial mycetoma: Diverse clinical presentations

    Directory of Open Access Journals (Sweden)

    Sharma Nand

    2008-01-01

    Full Text Available Nocardia spp are gram-positive, aerobic, acid-fast bacteria which exist as saprophytes in nature. Invasive disseminated infections are particularly common in immunocompromised or debilitated hosts. Superficial infections with Nocardia spp occur as a result of local trauma and contamination of the wound. Clinically, it presents as acute infection (abscesses or cellulitis, mycetoma, or sporotrichoid infection. Differential diagnosis includes eumycetoma, chromomycosis, blastomycosis, coccidioidomycosis, sporotrichosis, tuberculosis, botryomycosis, syphilis, yaws, and neoplasia. Its diagnosis is confirmed by demonstrating the causative organism in exudates (as granules, tissue specimens, or cultures. Early diagnosis will obviate need for drastic surgical measures as early institution of chemotherapy is effective in most patients. However, its diagnosis is often delayed due to diverse clinical presentations and for want of clinical suspicion, particularly in non-endemic areas. This paper presents 4 clinical forms of this not so uncommon disease, emphasizing the importance of high index of clinical suspicion, especially in non-endemic regions; and the significance of repeated examination of exudates for Nocardia granules for an early diagnosis.

  9. Industry Presentation to Regulatory Workshop

    International Nuclear Information System (INIS)

    Treasure, Mark

    2012-01-01

    Mr. Mark Treasure from EDF NGL gave a presentation on industry perspectives on safety culture oversight. Mr. Treasure is the Nuclear Inspection and Oversight Manager within the Safety and Regulation Division. The presentation started with an explanation of the role of the nuclear inspection and oversight group (internal regulator), and their current approach to internal oversight of safety culture. A key element of the current internal regulatory oversight program is Management and Leadership Assessments. These are carried out by a team including management peers from other plants to enhance credibility. Findings can be linked to safety performance, and typically identify issues in areas such as accountability arrangements and strategic focus of the leadership team. Safety indicators have also been introduced to show trends in safety management and safety performance for each EDF UK nuclear power plant. A periodic nuclear safety culture survey is also carried out to identify focus areas and progress. The presentation included discussion on views of the role of the nuclear regulator. Important aspects were identified as: - Supporting the internal regulator by seeking to understand before taking enforcement action, maintaining an open dialogue and recognising that this area is complex and that there is rarely a 'silver bullet' solution. - Communication: being visible and actively discussing safety improvement, and use of language which emphasises nuclear safety rather than legal compliance. - Positive reinforcement to recognise efforts and encourage further improvement

  10. XPL the Extensible Presentation Language

    Directory of Open Access Journals (Sweden)

    A. Santangelo

    2009-01-01

    Full Text Available The last decade has witnessed a growing interest in the development of web interfaces enabling both multiple ways to access contents and, at the same time, fruition by multiple modalities of interaction (point-and-click, contents reading, voice commands, gestures, etc.. In this paper we describe a framework aimed at streamlining the design process of multi-channel, multimodal interfaces enabling full reuse of software components. This framework is called the eXtensible Presentation architecture and Language (XPL, a presentation language based on design pattern paradigm that keeps separated the presentation layer from the underlying programming logic. The language supplies a methodology to expedite multimodal interface development and to reduce the effort to implement interfaces for multiple access devices, by means of using the same code. This paper describes a methodology approach based on Visual Design Pattern (ViDP and Verbal Design Pattern (VeDP, offering examples of multimodal and multichannel interfaces created with the XPL Editor.

  11. Quantum technology past, present, future: quantum energetics (Conference Presentation)

    Science.gov (United States)

    Choi, Sang H.

    2017-04-01

    Since the development of quantum physics in the early part of the 1900s, this field of study has made remarkable contributions to our civilization. Some of these advances include lasers, light-emitting diodes (LED), sensors, spectroscopy, quantum dots, quantum gravity and quantum entanglements. In 1998, the NASA Langley Research Center established a quantum technology committee to monitor the progress in this area and initiated research to determine the potential of quantum technology for future NASA missions. The areas of interest in quantum technology at NASA included fundamental quantum-optics materials associated with quantum dots and quantum wells, device-oriented photonic crystals, smart optics, quantum conductors, quantum information and computing, teleportation theorem, and quantum energetics. A brief review of the work performed, the progress made in advancing these technologies, and the potential NASA applications of quantum technology will be presented.

  12. Diabetic Myonecrosis: An Atypical Presentation

    Directory of Open Access Journals (Sweden)

    José Hernán Martínez

    2013-01-01

    Full Text Available Diabetic myonecrosis is a frequently unrecognized complication of longstanding and poorly controlled diabetes mellitus. The clinical presentation is swelling, pain, and tenderness of the involved muscle, most commonly the thigh muscles. Management consists of conservative measures including analgesia and rest. Short-term prognosis is good, but long-term prognosis is poor with most patients dying within 5 years. Failure to properly identify this condition will expose the patient to aggressive measures that could result in increased morbidity. To our knowledge this is the first case reported in which there was involvement of multiple muscle groups including upper and lower limbs.

  13. Variable retinal presentations in nanophthalmos

    International Nuclear Information System (INIS)

    Khan, A.; Zafar, S.N.

    2009-01-01

    Nanophthalmos is an uncommon developmental ocular disorder characterized by a small eye with short axial length, high hyperopia and high lens/eye volume ratio due to arrested development of the globe in all directions. Different types of fundus changes can rarely occur with nanophthalmos. We describe five cases of nanophthalmos, each of them presenting with a different fundus appearance. Our case series highlights variability of pigmentary changes from retinal flecks to bone spicules and bull's eye maculopathy, which are rare in the combinations described here. (author)

  14. Present art of reactivity determination

    International Nuclear Information System (INIS)

    Kaneko, Yoshihiko; Nakano, Masafumi; Matsuura, Shojiro

    1977-01-01

    Experimental techniques for reactivity determination of a reactor have been one of the long standing subjects in reactor physics. Recently, such a requirement was proposed by the reactor designers and operators that the values of reactivity should be measured more accurately. This is because importance is emphasized for the role of reactivity to the performance of reactor safety, economics and operability. Motivated by the requirement, some remarkable progresses are being made for the improvement of the experimental techniques. Then, the present review summarizes the research activities on this subject, identifies several reactor physics problems to be overcome, and makes mention of the future targets. (auth.)

  15. Addison's disease presenting with perimyocarditis.

    Science.gov (United States)

    Baranski Lamback, Elisa; Morandi, Grazia; Rapti, Eleni; Christov, Georgi; Brogan, Paul A; Hindmarsh, Peter

    2018-01-26

    Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association. A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA. The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Autoimmune adrenal insufficiency should be considered in all cases of pericarditis and perimyocarditis, especially when the severity of clinical manifestations exceeds the expected for the severity of the cardiac findings, as timely identification and prompt treatment may be life-saving.

  16. Metastatic liposarcoma present in mediastinum

    International Nuclear Information System (INIS)

    Vazquez Gonzalez, Jose Manuel; Danta Fundora, Debora; Collado Otero, Juan Carlos; Paredes Lopez, Dagmar I

    2009-01-01

    Authors present the case of a woman aged 59 operated on in three occasions due to the presence of neck nodules. Biopsy from two of them informs that be about a pleomorphous liposarcoma. After 6 years she came to consultation by a picture of high retrosternal pain and slight dyspnea. Thorax tomography shows a solid mass in anterior mediastinum. We made exeresis of a 10 cm diameter soft solid mass. Pathological Anatomy Service reported on relapse of a pleomorphous liposarcoma. In general, the postoperative course was satisfactory. Literature reviewed shows that the surgical treatment is the more used procedure in these cases. (author)

  17. Presentation at Innoventure Annual Competition

    Energy Technology Data Exchange (ETDEWEB)

    None

    2010-03-31

    This report documents the components of the workshop presented at the recent annual competition for the Innoventure program. The goal of the workshop was to focus on the delivery of science, technology, engineering, and math (STEM) concepts in a hands-on experiential learning format to increase interest in national security careers at NNSA, most of which are in the STEM fields. This work is a part of the National Security Preparedness Project (NSPP), being performed under a Department of Energy (DOE)/National Nuclear Security Administration (NNSA) grant.

  18. Vitality of optical vortices (Presentation)

    CSIR Research Space (South Africa)

    Roux, FS

    2014-02-01

    Full Text Available stream_source_info Roux3_2014.pdf.txt stream_content_type text/plain stream_size 3018 Content-Encoding UTF-8 stream_name Roux3_2014.pdf.txt Content-Type text/plain; charset=UTF-8 Title Vitality of optical vortices F Stef... Roux Presented at Complex Light and Optical Force VIII SPIE Photonics West 2014 Moscone Center, San Francisco, California USA 5 February 2014 CSIR National Laser Centre, Pretoria, South Africa – p. 1/11 Speckle Amplitude Phase – p. 2/11 Vortex...

  19. Cartography past, present and future

    CERN Document Server

    Rhind, DW

    2013-01-01

    Making maps dates back at least four thousand years and it is widely recognised that many maps are of great historical value and present a skilled method of summarising the real world on a sheet of paper. Less well known is the judgement involved in the selection and simplification of features, the complex transformation of space and the exacting standards which are needed in cartography. This book is primarily a tribute to Professor F.J. Ormeling, former President and Secretary/Treasurer of the ICA and gives a wide ranging review of the current status of cartography, how this status was atta

  20. STS-95 Post Flight Presentation

    Science.gov (United States)

    1998-01-01

    The STS-95 flight crew, Cmdr. Curtis L. Brown, Pilot Steven W. Lindsey, Mission Specialists Scott E. Parazynski, Stephen K. Robinson, and Pedro Duque, and Payload Specialists Chiaki Mukai and John H. Glenn present a video mission over-view of their space flight. Images include prelaunch activities such as eating the traditional breakfast, crew suit-up, and the ride out to the launch pad. Also, included are various panoramic views of the shuttle on the pad. The crew can be seen being readied in the "whiteroom" for their mission. After the closing of the hatch and arm retraction, launch activities are shown including countdown, engine ignition, launch, and the separation of the Solid Rocket Boosters. The primary objectives, which include the conducting of a variety of science experiments in the pressurized SPACEHAB module, the deployment and retrieval of the Spartan free-flyer payload, and operations with the HST Orbiting Systems Test (HOST) and the International Extreme Ultraviolet Hitchhiker (IEH) payloads are discussed in both the video and still photo presentation.

  1. OROPHARYNGEAL TUBERCULOSIS: AN UNUSUAL PRESENTATION

    Directory of Open Access Journals (Sweden)

    M H Dadgarnia

    2008-12-01

    Full Text Available "nTuberculosis (TB still represents a major public health problem worldwide. The primary form of disease is most often localized to the lung. In a minority of patients, progressive pulmonary disease spreads to other organ systems through self inoculation via infected sputum, blood and lymphatic system, establishing the secondary form of tuberculosis. We present a patient that was referred to us with complaint of ulcerative mouth lesions from 3 months ago. In physical examination multiple erythematous and irregularly ulcerative lesions affecting soft palate area, uvula and anterior tonsillar pillar was noted bilaterally. Punch biopsy was done from several points that revealed chronic granulomatous inflammation. Ziehl-Nielsen staining of cultured specimen demonstrated acid-fast bacilli. Chest X-ray showed apical pulmonary involvement, suggesting tuberculosis infection. Patient was treated with anti-tuberculosis 4 drugs regimen. In the one year follow-up period after complete treatment; patient didn't have any evidence of disease. Oral and oropharyngeal TB lesions are uncommon, it is estimated that only 0.05-5% of total TB cases may present with oral manifestations, but should be an important consideration in the differential diagnosis of lesions that appear in the oral cavity and oropharynx. The secondary form is more frequent and involves mainly the tongue but involvement of pharynx is quite rare condition. Although tuberculosis of oropharynx is relatively rare, with the increasing incidence of tuberculosis, it must be considered in the differential diagnosis of atypical ulcerative lesions of the mouth and oropharynx.

  2. Early presentation of primary glioblastoma.

    Science.gov (United States)

    Faguer, R; Tanguy, J-Y; Rousseau, A; Clavreul, A; Menei, P

    2014-08-01

    Clinical and neuroimaging findings of glioblastomas (GBM) at an early stage have rarely been described and those tumors are most probably under-diagnosed. Furthermore, their genetic alterations, to our knowledge, have never been previously reported. We report the clinical as well as neuroimaging findings of four early cases of patients with GBM. In our series, early stage GBM occurred at a mean age of 57 years. All patients had seizures as their first symptom. In all early stages, MRI showed a hyperintense signal on T2-weighted sequences and an enhancement on GdE-T1WI sequences. A hyperintense signal on diffusion sequences with a low ADC value was also found. These early observed occurrences of GBM developed rapidly and presented the MRI characteristics of classic GBM within a few weeks. The GBM size was multiplied by 32 in one month. Immunohistochemical analysis indicated the de novo nature of these tumors, i.e. absence of mutant IDH1 R132H protein expression, which is a diagnostic marker of low-grade diffuse glioma and secondary GBM. A better knowledge of early GBM presentation would allow a more suitable management of the patients and may improve their prognosis. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  3. Teratology - past, present and future.

    Science.gov (United States)

    Ujházy, Eduard; Mach, Mojmír; Navarová, Jana; Brucknerová, Ingrid; Dubovický, Michal

    2012-12-01

    Teratology is the science that studies the causes, mechanisms, and patterns of abnormal development. The authors present an updated overview of the most important milestones and stages of the development of modern teratology. Development of knowledge and society led to the recognition that causes of congenital developmental disorders (CDDs) might be caused by various mechanical effects, foetal diseases, and retarded or arrested development of the embryo and foetus. Based on the analysis of the historical development of hypotheses and theories representing a decisive contribution to this field, we present a survey of the six Wilson's fundamental principles of teratology. The aim of observing these principles is to get insight into developmental relations and to understand mechanisms of action on the level of cell populations (elementary morphogenetic processes), tissues and organs. It is important to realise that any negative intervention into the normal course of these processes, either on genetic or non-genetic basis, inevitably leads to a sequence of subsequent changes resulting in CDDs. Moreover, the classical toxicologic monotonic dose-response paradigm recently has been challenged by the so-called "low dose-hypothesis", particularly in the case of endocrine active substances. These include some pesticides, dioxins, polychlorobiphenyls (PCBs), and bisphenol A. Despite modern approaches of molecular biology and genetics, along with top diagnostic techniques, we are still not able to identify the actual cause in more than 65 to 70% of all congenital defects classified as having an unknown etiology. Today CDDs include any birth defect, either morphological, biochemical, or behavioural.

  4. Cutaneous sporotrichosis: Unusual clinical presentations

    Directory of Open Access Journals (Sweden)

    Mahajan Vikram

    2010-01-01

    Full Text Available Three unusual clinical forms of sporotrichosis described in this paper will be a primer for the clinicians for an early diagnosis and treatment, especially in its unusual presentations. Case 1, a 52-year-old man, developed sporotrichosis over pre-existing facial nodulo-ulcerative basal cell carcinoma of seven-year duration, due to its contamination perhaps from topical herbal pastes and lymphocutaneous sporotrichosis over right hand/forearm from facial lesion/herbal paste. Case 2, a 25-year-old woman, presented with disseminated systemic-cutaneous, osteoarticular and possibly pleural (effusion sporotrichosis. There was no laboratory evidence of tuberculosis and treatment with anti-tuberculosis drugs (ATT did not benefit. Both these cases were diagnosed by histopathology/culture of S. schenckii from tissue specimens. Case 3, a 20-year-old girl, had multiple intensely pruritic, nodular lesions over/around left knee of two-year duration. She was diagnosed clinically as a case of prurigo nodularis and histologically as cutaneous tuberculosis, albeit, other laboratory investigations and treatment with ATT did not support the diagnosis. All the three patients responded well to saturated solution of potassium iodide (SSKI therapy. A high clinical suspicion is important in early diagnosis and treatment to prevent chronicity and morbidity in these patients. SSKI is fairly safe and effective when itraconazole is not affordable/ available.

  5. Presentation Extensions of the SOAP

    Science.gov (United States)

    Carnright, Robert; Stodden, David; Coggi, John

    2009-01-01

    A set of extensions of the Satellite Orbit Analysis Program (SOAP) enables simultaneous and/or sequential presentation of information from multiple sources. SOAP is used in the aerospace community as a means of collaborative visualization and analysis of data on planned spacecraft missions. The following definitions of terms also describe the display modalities of SOAP as now extended: In SOAP terminology, View signifies an animated three-dimensional (3D) scene, two-dimensional still image, plot of numerical data, or any other visible display derived from a computational simulation or other data source; a) "Viewport" signifies a rectangular portion of a computer-display window containing a view; b) "Palette" signifies a collection of one or more viewports configured for simultaneous (split-screen) display in the same window; c) "Slide" signifies a palette with a beginning and ending time and an animation time step; and d) "Presentation" signifies a prescribed sequence of slides. For example, multiple 3D views from different locations can be crafted for simultaneous display and combined with numerical plots and other representations of data for both qualitative and quantitative analysis. The resulting sets of views can be temporally sequenced to convey visual impressions of a sequence of events for a planned mission.

  6. Bilirubin present in diverse angiosperms.

    Science.gov (United States)

    Pirone, Cary; Johnson, Jodie V; Quirke, J Martin E; Priestap, Horacio A; Lee, David

    2010-01-01

    Bilirubin is an orange-yellow tetrapyrrole produced from the breakdown of heme by mammals and some other vertebrates. Plants, algae and cyanobacteria synthesize molecules similar to bilirubin, including the protein-bound bilins and phytochromobilin which harvest or sense light. Recently, we discovered bilirubin in the arils of Strelitzia nicolai, the White Bird of Paradise Tree, which was the first example of this molecule in a higher plant. Subsequently, we identified bilirubin in both the arils and the flowers of Strelitzia reginae, the Bird of Paradise Flower. In the arils of both species, bilirubin is present as the primary pigment, and thus functions to produce colour. Previously, no tetrapyrroles were known to generate display colour in plants. We were therefore interested in determining whether bilirubin is broadly distributed in the plant kingdom and whether it contributes to colour in other species. In this paper, we use HPLC/UV and HPLC/UV/electrospray ionization-tandem mass spectrometry (HPLC/UV/ESI-MS/MS) to search for bilirubin in 10 species across diverse angiosperm lineages. Bilirubin was present in eight species from the orders Zingiberales, Arecales and Myrtales, but only contributed to colour in species within the Strelitziaceae. The wide distribution of bilirubin in angiosperms indicates the need to re-assess some metabolic details of an important and universal biosynthetic pathway in plants, and further explore its evolutionary history and function. Although colour production was limited to the Strelitziaceae in this study, further sampling may indicate otherwise.

  7. Anaphylactic reactions presenting with hypertension.

    Science.gov (United States)

    Solmazgul, Emrullah; Kutlu, Ali; Dogru, Salim; Ozalper, Veysel; Cetindagli, Ibrahim; Sezer, Ogun; Salmanoglu, Musa; Kilic, Erol; Karabacak, Ercan; Ozturk, Sami

    2016-01-01

    Although a few case reports about hypertensive anaphylaxis (HA) are available in the present literature, there is no study about the prevalence of HA. In this study, we review our cases with anaphylaxis presenting with hypertension and ascertain its prevalence. The documents of the patients who had anaphylactic reactions after the procedures performed for the diagnosis and treatment of allergic diseases in GATA Haydarpasa Clinic of Allergy and Immunology between January 2010 and December 2014 were retrospectively reviewed. Within the study period, 324 patients had undergone 4332 procedures in which 62 of them had developed anaphylaxis. During the procedures, the rate of anaphylaxis was found to be 1.43 %. The rate of HA among the anaphylaxis patients was 12.9 % (8 of 62 patients). During treatments, 2 patients received adrenaline injections without any adverse reaction. HA may be seen at a considerable rate during an anaphylactic reaction. Anaphylaxis and hypertension can be recovered by adrenaline injection when required. According to the best of our knowledge, this study is the first original study about the prevalence of HA in English-language medical literature.

  8. Evaluation of patients undergoing lung volume reduction surgery: Ancillary information available from computed tomography

    International Nuclear Information System (INIS)

    Cleverley, Joanne R.; Desai, Sujal R.; Wells, Athol U.; Koyama, Hiroshi; Eastick, Sian; Schmidt, Maria A.; Charrier, Clare L.; Gatehouse, Peter D.; Goldstraw, Peter; Pepper, John R.; Geddes, Duncan M.; Hansell, David M.

    2000-01-01

    AIM: A number of imaging techniques have been used for the pre-operative assessment of patients for lung volume reduction surgery (LVRS). We evaluated whether data currently acquired from perfusion scintigrams and cine MR of the diaphragm are obtainable from high resolution CT (HRCT) of the thorax. MATERIALS AND METHODS: Thirty patients taking part in a randomized controlled trial of LVRS against maximal medical therapy were evaluated. HRCT examinations (n= 30) were scored for (i) the extent and distribution of emphysema; (ii) the extent of normal pulmonary vasculature; and (iii) diaphragmatic contour, apparent defects and herniation. On scintigraphy, (n28), perfusion of the lower thirds of both lungs, as a proportion of total lung perfusion (LZ/T PERF ), was expressed as a percentage of predicted values (derived from 10 normal control subjects). On cine MR (n= 25) hemidiaphragmatic excursion and coordination were recorded. RESULTS: Extensive emphysema was present on HRCT (60% ± 13.2%). There was strong correlation between the extent of normal pulmonary vasculature on HRCT and on perfusion scanning (r s = 0.85, P< 0.00005). Hemidiaphragmatic incoordination on MR was weakly associated with hemidiaphragmatic eventration on HRCT (P0.04). CONCLUSION: The strong correlation between lung perfusion assessed by HRCT and lung perfusion on scintigraphy suggests that perfusion scintigraphy is superfluous in the pre-operative evaluation of patients with emphysema for LVRS. Cleverley, J.R. (2000)

  9. Uncommon presentation of pulmonary aspergilloma

    Directory of Open Access Journals (Sweden)

    Baradkar V

    2009-01-01

    Full Text Available Cases of pulmonary aspergilloma without any predisposing factors are rarely reported. Clinical presentation varies from case to case. Here, we report a case of pulmonary aspergilloma in a 60-year-old male patient who was admitted to the Intensive Respiratory Care Unit with spontaneous pneumothorax. The patient had a history of dyspnea on exertion since 9 months and mild haemoptysis since the last 6 months. A computerised tomographic scan of the lungs showed a lesion in the left main bronchus along with obstructive emphysema of the right lung, moderate pneumothorax and mediastinal emphysema. Bronchoscopy was performed and the biopsy samples were processed for histopathological examination and culture on Sabouraud′s dextrose agar, which yielded growth of Aspergillus flavus. Repeat sputum samples also yielded the growth of A. flavus . The patient responded to intravenous liposomaamphotericin B and intercostal drainage.

  10. Site selection: Past and present

    International Nuclear Information System (INIS)

    Tilford, N.R.

    1994-01-01

    Site selection has been going on since the earliest times. The process has evolved through the Industrial Revolution to the present period of exploding population and environmental awareness. Now the work must be done both with increasing sophistication and greater transparency. Modern techniques for site selection have been developed during the last two decades or so, utilizing a teachable body of knowledge and a growing literature. Many firms and individuals have contributed to this growing field. The driving force has been the need for such a process in siting and licensing of critical facilities such as nuclear power plants. A list of crucial, documented steps for identifying social impacts and acceptability are provided. A recent innovation is the self-selection method developed by government. The Superconducting Supercollider serves as an example of this approach. Geological or geologically dependent factors often dominate the process. The role as engineering and environmental geoscientists is to provide responsible leadership, consultation, and communication to the effort

  11. Neurocysticercosis presenting as pseudobulbar palsy

    Directory of Open Access Journals (Sweden)

    Arinaganahalli Subbanna Praveen Kumar

    2014-01-01

    Full Text Available Neurocysticercosis (NCC is the most common helminthic infestation of the central nervous system (CNS and a leading cause of acquired epilepsy worldwide. The common manifestations of NCC are seizures and headache. The NCC as a cause of pseudobulbar palsy is very unusual and not reported yet in the literature. A pseudobulbar palsy can occur in any disorder that causes bilateral corticobulbar disease. The common etiologies of pseudobulbar palsy are vascular, demyelinative, or motor neuron disease. We report a 38-year-old female patient who presented with partial seizures and pseudobulbar palsy. The MRI brain showed multiple small cysts with scolex in both the cerebral hemispheres and a giant intraparenchymal cyst. Our patient responded well to standard treatment of neurocysticercosis and antiepileptics.

  12. Present challenges in hadrontherapy techniques

    Science.gov (United States)

    Amaldi, U.; Braccini, S.

    2011-07-01

    Hadrontherapy is a high-precision technique in cancer radiation therapy, which allows obtaining a superior conformal treatment with respect to photons used in conventional radiation therapy. To reach this ambitious goal without reducing the patient throughput needed in a hospital-based environment, the physical and radiobiological properties of charged hadrons, protons and carbon ions in particular, have to be exploited at best, making use of the most modern technologies issued from research in nuclear and particle physics. In the present days, we are assisting to a continuous technological challenge, leading to the conception and to the development of innovative methods and instruments. In this paper, the most relevant challenges in dose delivery systems, gantries, imaging, quality assurance and particle accelerators are reviewed.

  13. Ebolavirus Evolution: Past and Present.

    Science.gov (United States)

    de La Vega, Marc-Antoine; Stein, Derek; Kobinger, Gary P

    2015-01-01

    The past year has marked the most devastating Ebola outbreak the world has ever witnessed, with over 28,000 cases and over 11,000 deaths. Ebola virus (EBOV) has now been around for almost 50 years. In this review, we discuss past and present outbreaks of EBOV and how those variants evolved over time. We explore and discuss selective pressures that drive the evolution of different Ebola variants, and how they may modify the efficacy of therapeutic treatments and vaccines currently being developed. Finally, given the unprecedented size and spread of the outbreak, as well as the extended period of replication in human hosts, specific attention is given to the 2014-2015 West African outbreak variant (Makona).

  14. Ebolavirus Evolution: Past and Present.

    Directory of Open Access Journals (Sweden)

    Marc-Antoine de La Vega

    Full Text Available The past year has marked the most devastating Ebola outbreak the world has ever witnessed, with over 28,000 cases and over 11,000 deaths. Ebola virus (EBOV has now been around for almost 50 years. In this review, we discuss past and present outbreaks of EBOV and how those variants evolved over time. We explore and discuss selective pressures that drive the evolution of different Ebola variants, and how they may modify the efficacy of therapeutic treatments and vaccines currently being developed. Finally, given the unprecedented size and spread of the outbreak, as well as the extended period of replication in human hosts, specific attention is given to the 2014-2015 West African outbreak variant (Makona.

  15. Tritium practices past and present

    International Nuclear Information System (INIS)

    Gede, V.P.; Gildea, P.D.

    1980-01-01

    History of the production and use of tritium, as well as handling techniques, are reviewed. Handling techniques first used at Lawrence Livermore National Laboratory made use of glass vacuum systems and relatively crude ion chambers for monitoring airborne activity. The first use of inert atmosphere glove boxes demonstrated that uptake through the skin could be a serious personnel exposure problem. Growing environmental concerns in the early 1970's resulted in the implementation by the Atomic Energy Commission of a new criteria to limit atmospheric tritium releases to levels as low as practicable. An important result of the new criteria was the development of containment and recovery systems to capture tritium rather than vent it to the atmosphere. The Sandia National Laboratories, Livermore, Tritium Research Laboratory containment and decontamination systems are presented as a typical example of this technology. The application of computers to control systems is expected to provide the greatest potential for change in future tritium handling practices

  16. Speaker's presentations. Energy supply security

    International Nuclear Information System (INIS)

    Pierret, Ch.

    2000-01-01

    This document is a collection of most of the papers used by the speakers of the European Seminar on Energy Supply Security organised in Paris (at the French Ministry of Economy, Finance and Industry) on 24 November 2000 by the General Direction of Energy and Raw Materials, in co-operation with the European Commission and the French Planning Office. About 250 attendees were present, including a lot of high level Civil Servants from the 15 European State members, and their questions have allowed to create a rich debate. It took place five days before the publication, on 29 November 2000, by the European Commission, of the Green Paper 'Towards a European Strategy for the Security of Energy Supply'. This French initiative, which took place within the framework of the European Presidency of the European Union, during the second half-year 2000. will bring a first impetus to the brainstorming launched by the Commission. (author)

  17. Different presentations of pulmonary criptococosis

    International Nuclear Information System (INIS)

    Londono Palacio, Natalia; Ojeda Paulina; Ortiz, Guillermo; Alwers Rolf C; Munive, Abraham Ali

    1998-01-01

    We presented 4 cases studies of criptococosis, all of them with pulmonary compromise, diagnosed at Hospital Santa Clara in a period of 5 years. It's important to note that none of the patients were HIV positive. In the first case studied the cellular immune compromise has, a multifactorial origin: rheumatoid arthritis and the administration of corticoids. In the second case studied, SLE can explain its origin. In the third case studied, because of the short hospital stay, the investigation with respect to the etiology of the immune-compromise was limited. In the fourth case studied, because of a low CD4 count Elisa immunoassay for HIV was repeated without its confirmation. Spontaneous recuperation was obtained, being free of infection for a period of five years

  18. Director general presentation to personnel

    CERN Multimedia

    2016-01-01

    Dear Colleagues, Many important discussions are scheduled for the upcoming Council Week (13-17 June) on topics including the Medium-Term Plan, the Pension Fund and other matters of great relevance to us.   I would therefore like to share the main outcome of the week with you and I invite you to join me and the Directors in the Main Auditorium at 10 a.m. on Thursday 23 June. The meeting will last about one hour and a webcast will also be available. Best regards, Fabiola Gianotti DG presentation to personnel Thursday 23 June at 10 am Main Auditorium Retransmission in Council Chamber, IT Auditorium, Kjell Jonhsen Auditorium, Prevessin 864-1-C02 Webcast on cern.ch/webcast More information on the event page.

  19. Atypical presentations of celiac disease

    Directory of Open Access Journals (Sweden)

    Balasa Adriana Luminita

    2016-08-01

    Full Text Available In this study we evaluated the association of celiac disease in 81 children with autoimmune disease and genetic syndromes over a two years periods (January 2014 to July 2016 in Pediatric Clinic in Constanta. Because the extraintestinal symptoms are an atypical presentation of celiac disease we determined in these children the presence of celiac disease antibodies: Anti-tissue Transglutaminase Antibody IgA and IgA total serum level as a screening method followeds in selective cases by Anti-tissue Transglutaminase Antibody IgG, anti-endomysial antibodies, deamidated gliadin antibodies IgA and IgG and intestinal biopsia. In our study 8 patients had been diagnosed with celiac disease with extraintestinal symptoms, of which 4 with type 1 diabetes, 1 patient with ataxia, 2 patients with dermatitis herpetiformis and 1 patient with Down syndrome that associate also autoimmune thyroiditis, alopecia areata, enamel hypoplasia.

  20. Oropharyngeal styloids: An unusual presentation

    Directory of Open Access Journals (Sweden)

    Lathadevi Hassan Thotappa

    2012-01-01

    Full Text Available The close proximity of the styloid process to many of the vital neurovascular structures in the neck makes it clinically significant. The styloid process is said to be elongated if it is longer than 3.0 cm in length. Anatomical variations are very common and clinical symptoms arising from such variations have to be recognized. Elongated styloid processes may cause chronic throat pain along with foreign body sensation, dysphagia, vague facial pain, and otalgia. Surgical excision of an elongated styloid is considered as a satisfactory treatment for such cases. Here, we present a unique case of bilaterally elongated styloids that could be visualized just by depressing the tongue, when they appeared like the tusks of an elephant in the oropharyngeal region.

  1. Splenic abscess: a rare presentation

    Directory of Open Access Journals (Sweden)

    Mohit Bhatia

    2015-01-01

    Full Text Available Splenic abscess is a rare clinical entity with an incidence of 0.2-0.7% in autopsy-based studies. When untreated, splenic abscess is associated with nearly 100% mortality; in treated patients, the mortality rate is 16.6% during the first 90 days. It mostly occurs in patients with neoplasia, immunodeficiency, trauma, diabetes or splenic infarct. The incidence of splenic abscess is thought to be growing because of the increase in the number of immunocompromised patients who are particularly at risk for this disease and also because of the widespread use of diagnostic modalities. However, the optimal treatment for this remains unclear. We present a case of a 42-year-old man diagnosed with multiloculated splenic abscess and was subjected to splenectomy.

  2. Safeguards instrumentation: past, present, future

    International Nuclear Information System (INIS)

    Higinbotham, W.A.

    1982-01-01

    Instruments are essential for accounting, for surveillance and for protection of nuclear materials. The development and application of such instrumentation is reviewed, with special attention to international safeguards applications. Active and passive nondestructive assay techniques are some 25 years of age. The important advances have been in learning how to use them effectively for specific applications, accompanied by major advances in radiation detectors, electronics, and, more recently, in mini-computers. The progress in seals has been disappointingly slow. Surveillance cameras have been widely used for many applications other than safeguards. The revolution in TV technology will have important implications. More sophisticated containment/surveillance equipment is being developed but has yet to be exploited. On the basis of this history, some expectations for instrumentation in the near future are presented

  3. Cavernous hemangioma presenting marked hyperostosis

    International Nuclear Information System (INIS)

    Kobata, Hitoshi; Miyake, Hiroji; Kitamura, Junji; Kajikawa, Hiroshi; Ohta, Tomio

    1988-01-01

    The authors report here a case of hemangioma of the left parietal bone which presented headache and papilledema. This patient is a 37-year-old female who had, prior to admission, complained of increasing headache for one year and blurred vision for three months. She had no history of head injury. Local physical examinations revealed a slight bulging in her left parietal region which was insensitive to palpation and not adherent to the overlying scalp. Neurological examinations revealed bilateral papilledema and an incongruous bitemporal upper quadrant defect in the visual field. All the other neurological and laboratory data were normal. A plain skull roentogenogram showed a 9 x 9 cm osteolytic and characteristic honeycomb lesion in the parietal region. Systemic bone survey revealed a similar lesion in the right tibia which was not histologically examined. A marked accumulation of isotopes was detected on the bone scintigrams at both lesions. Selective external carotid angiograms demonstrated a tumor stain fed by the superficial temporal, occipital, and middle meningial arteries. CT scans of the brain and skull clearly showed a local thickening of and structural changes in the skull bone and also a mass effect on the brain and lateral ventricle. The lesioned bone was removed en bloc and replaced by an artificial bone. It was highly vascular, but not adherent to the overlying dura. The post-operative course was uneventful, and the headache and papilledema disappeared. Hemangioma of the skull presenting marked hyperostosis, as reported above, seems to be rare. In addition, in this case, skeletal angioma without any clinical manifestation was detected. Clinical and radiological pictures of the hemangioma of the skull and other bones were briefly discussed. (author)

  4. Amebiasis Presenting as Pleuropulmonary Disease

    Science.gov (United States)

    Kubitschek, Kenneth R.; Peters, Jay; Nickeson, David; Musher, Daniel M.

    1985-01-01

    Seven patients with amebic liver abscess presenting as pleuropulmonary disease were admitted to hospital initially because of pulmonary symptoms and were found to have amebic liver disease. Three categories of pleuropulmonary involvement included reactive inflammation of the pleura or lung, rupture of a hepatic abscess into the pleural space and rupture of a hepatic abscess into the bronchial airways. The preferred medical treatment is with metronidazole, but rupture of hepatic amebic abscess into the pleural space requires drainage in addition to medical therapy. In contrast, rupture into the bronchus may provide spontaneous drainage so that only medical therapy is needed. Recovery from amebiasis in all three categories is generally complete. Morbidity and mortality increase with failure to correctly identify amebic infection of the liver as the underlying cause. Because, in new cases, no findings specifically suggest that pleuropulmonary disease is a complication of hepatic amebic abscess, this possibility needs to be considered, especially in persons who are at risk of having been infected with amebae. ImagesFigure 1.Figure 2. PMID:4013249

  5. Surface Disinfections: Present and Future

    Directory of Open Access Journals (Sweden)

    Matteo Saccucci

    2018-01-01

    Full Text Available The propagation of antibiotic resistance increases the chances of major infections for patients during hospitalization and the spread of health related diseases. Therefore finding new and effective solutions to prevent the proliferation of pathogenic microorganisms is critical, in order to protect hospital environment, such as the surfaces of biomedical devices. Modern nanotechnology has proven to be an effective countermeasure to tackle the threat of infections. On this note, recent scientific breakthroughs have demonstrated that antimicrobial nanomaterials are effective in preventing pathogens from developing resistance. Despite the ability to destroy a great deal of bacteria and control the outbreak of infections, nanomaterials present many other advantages. Moreover, it is unlikely for nanomaterials to develop resistance due to their multiple and simultaneous bactericidal mechanisms. In recent years, science has explored more complex antimicrobial coatings and nanomaterials based on graphene have shown great potential in antibacterial treatment. The purpose of this article is to deepen the discussion on the threat of infections related to surface disinfection and to assess the state of the art and potential solutions, with specific focus on disinfection procedures using nanomaterials.

  6. Database Systems - Present and Future

    Directory of Open Access Journals (Sweden)

    2009-01-01

    Full Text Available The database systems have nowadays an increasingly important role in the knowledge-based society, in which computers have penetrated all fields of activity and the Internet tends to develop worldwide. In the current informatics context, the development of the applications with databases is the work of the specialists. Using databases, reach a database from various applications, and also some of related concepts, have become accessible to all categories of IT users. This paper aims to summarize the curricular area regarding the fundamental database systems issues, which are necessary in order to train specialists in economic informatics higher education. The database systems integrate and interfere with several informatics technologies and therefore are more difficult to understand and use. Thus, students should know already a set of minimum, mandatory concepts and their practical implementation: computer systems, programming techniques, programming languages, data structures. The article also presents the actual trends in the evolution of the database systems, in the context of economic informatics.

  7. STS-70 Post Flight Presentation

    Science.gov (United States)

    Peterson, Glen (Editor)

    1995-01-01

    In this post-flight overview, the flight crew of the STS-70 mission, Tom Henricks (Cmdr.), Kevin Kregel (Pilot), Major Nancy Currie (MS), Dr. Mary Ellen Weber (MS), and Dr. Don Thomas (MS), discuss their mission and accompanying experiments. Pre-flight, launch, and orbital footage is followed by the in-orbit deployment of the Tracking and Data Relay Satellite (TDRS) and a discussion of the following spaceborne experiments: a microgravity bioreactor experiment to grow 3D body-like tissue; pregnant rat muscular changes in microgravity; embryonic development in microgravity; Shuttle Amateur Radio Experiment (SAREX); terrain surface imagery using the HERCULES camera; and a range of other physiological tests, including an eye and vision test. Views of Earth include: tropical storm Chantal; the Nile River and Red Sea; lightning over Brazil. A three planet view (Earth, Mars, and Venus) was taken right before sunrise. The end footage shows shuttle pre-landing checkout, entry, and landing, along with a slide presentation of the flight.

  8. A cool present for LEIR

    CERN Multimedia

    2005-01-01

    LEIR (Low Energy Ion Ring), which will supply lead ions to the LHC experiments, has taken delivery of one of its key components, its electron cooling system. From left to right, Gérard Tranquille, Virginia Prieto and Roland Sautier, in charge of the electron cooling system for LEIR at CERN, and Christian Lacroix, in charge of installation for the LEIR machine. On 16 December, the day before CERN's annual closure, the LEIR teams received a rather impressive Christmas present. The "parcel" from Russia, measuring 7 metres in length and 4 metres in height, weighed no less than 20 tonnes! The component will, in fact, be one of the key elements of the future LEIR, namely its electron cooling system. LEIR is one of the links in the injector chain that will supply lead ions to the LHC experiments, in particular ALICE (see Bulletin No. 28/2004 of 5 July 2004), within the framework of the I-LHC Project. The electron cooling system is designed to reduce and standardise transverse ion velocity. This focuses the bea...

  9. Present status of Tokamak research

    International Nuclear Information System (INIS)

    Basu, Jayanta

    1991-01-01

    The scenario of thermonuclear fusion research is presented, and the tokamak which is the most promising candidate as a fusion reactor is introduced. A brief survey is given of the most noteworthy tokamaks in the global context, and fusion programmes relating to Next Step devices are outlined. Supplementary heating of tokamak plasma by different methods is briefly reviewed; the latest achievements in heating to fusion temperatures are also reported. The progress towards the high value of the fusion product necessary for ignition is described. The improvement in plasma confinement brought about especially by the H-mode, is discussed. The latest situation in pushing up Β for increasing the efficiency of a tokamak is elucidated. Mention is made of the different types of wall treatment of the tokamak vessel for impurity control, which has led to a significant improvement in tokamak performance. Different methods of current drive for steady state tokamak operation are reviewed, and the issue of current drive efficiency is addressed. A short resume is given of the various diagnostic methods which are employed on a routine basis in the major tokamak centres. A few diagnostics recently developed or proposed in the context of the advanced tokamaks as well as the Next Step devices are indicated. The important role of the interplay between theory, experiment and simulation is noted, and the areas of investigation requiring concerted effort for further progress in tokamak research are identified. (author). 17 refs

  10. Presentation of suspected pediatric uveitis.

    Science.gov (United States)

    Saeed, Muhammad Usman; Raza, Syed Hamid; Goyal, Sudeshna; Cleary, Gavin; Newman, William David; Chandna, Arvind

    2014-01-01

    Presentation of suspected pediatric uveitis: Pediatric uveitis is usually managed in specialized ophthalmic centers in the UK. Meaningful data acquisition in these clinics may be helpful in clinical governance, and healthcare planning in a specialty that is gradually changing due to changes in treatment choices. Retrospective analysis of prospectively acquired data in the Liverpool pediatric uveitis database was performed. Analysis of our data, based on 147 patients, with a mean age of 10 years, indicated a female to male ratio of 2:1. 99% of patients were Caucasian. Our data indicates 86% of all patients attending the uveitis clinic were diagnosed with juvenile idiopathic arthritis, followed by intermediate uveitis 5% and idiopathic uveitis 4%. 46% of patients required treatment. Systemic treatment included methotrexate (34%), prednisolone (14%), etanercept (6%), ciclosporin (6%), mycophenolate (3%), and infliximab (1%). Severe visual loss (defined by counting fingers or below vision) was seen in 10 eyes despite appropriately treated chronic uveitis. Our data shows uveitis-related ocular morbidity in a predominantly pediatric Caucasian population. Patients with severe and chronic uveitis may experience significant uveitis-related complications and subsequent visual loss despite aggressive treatment.

  11. UNSCEAR: present and future activities

    International Nuclear Information System (INIS)

    Holm, Lars-Erik

    2000-01-01

    United Nations Scientific Committee on the Effects of Atomic Radiation (UNSCEAR) was established in 1955. The Committee reviews the exposure of the world population to all sources of radiation under normal circumstances as well as after accidents, and is also the prime international scientific body reviewing and assessing the health risks of exposure to ionizing radiation. Its estimates have been and are still being used by major international bodies including the ICRP, IAEA, and WHO. UNSCEAR's publications form the scientific basis on which international and national agencies develop appropriate radiation protection standards for workers, patients and the general public. UNSCEAR reports yearly to the General Assembly and submits regularly comprehensive reports with detailed scientific annexes on the sources and biological effects of ionizing radiation. In 1995, UNSCEAR began a new program of review of the sources, exposure and biological effects of ionizing radiation. A comprehensive report with scientific annexes will be published in the year 2000. The report will include exposures from natural and man-made radiation sources, occupational and medical radiation exposures, and dose assessment methodologies. The biological effects will cover epidemiological evaluation of radiation-induced cancer, DNA repair and mutagenesis, hereditary effects, combined effects of radiation and other agents, as well as models, mechanisms and uncertainties of biological effects at low radiation doses. An assessment of exposures and effects of the Chernobyl accidents is also made. A brief overview is presented here of the range of subjects considered by the Committee. (author)

  12. Exoplanets: Past, Present, and Future

    Directory of Open Access Journals (Sweden)

    Chien-Hsiu Lee

    2018-04-01

    Full Text Available Our understanding of extra-solar planet systems is highly driven by advances in observations in the past decade. Thanks to high precision spectrographs, we are able to reveal unseen companions to stars with the radial velocity method. High precision photometry from the space, especially with the Kepler mission, enables us to detect planets when they transit their stars and dim the stellar light by merely one percent or smaller. Ultra wide-field, high cadence, continuous monitoring of the Galactic bulge from different sites around the southern hemisphere provides us the opportunity to observe microlensing effects caused by planetary systems from the solar neighborhood, all the way to the Milky Way center. The exquisite AO imaging from ground-based large telescopes, coupled with high-contrast coronagraph, captured the photons directly emitted by planets around other stars. In this article, I present a concise review of the extra-solar planet discoveries, discussing the strengths and weaknesses of the major planetary detection methods, providing an overview of our current understanding of planetary formation and evolution given the tremendous observations delivered by various methods, as well as on-going and planned observation endeavors to provide a clear picture of extra-solar planetary systems.

  13. Presentation of the results 97

    International Nuclear Information System (INIS)

    1998-01-01

    Gaz de France (GDF) presents in a press kit the main results achieved in 1997. The European community has formulated a project of definition of rules concerning the natural gas market. Minima values for annual consumptions have been defined and the consumption and use of natural gas in EC countries is described. The financial results of GDF in 1997 are compared with previous years. Net sales grew by nearly 1 billion french francs to reach 55 billion. Overall sales were down by 4% but the share of natural gas in french energy consumption increased and sales to industrial users went up in particular due to new cogeneration plants installed in 1996. A comparison of gas prices in Europe is given for the years 1985 to 1998. The efforts of GDF to offer increased quality of services concerning in particular safety aspects as checking programs and installation of new safeguard appliances are described. The efforts of GDF to increase the energy market share of natural gas resulted in the development of new products like cogeneration, air conditioning and fuels for automotive needs. (C.B.)

  14. Nuclear Fuels: Present and Future

    Directory of Open Access Journals (Sweden)

    Donald R. Olander

    2009-02-01

    Full Text Available The important new developments in nuclear fuels and their problems are reviewed and compared with the status of present light-water reactor fuels. The limitations of these fuels and the reactors they power are reviewed with respect to important recent concerns, namely provision of outlet coolant temperatures high enough for use in H2 production, destruction of plutonium to eliminate proliferation concerns, and burning of the minor actinides to reduce the waste repository heat load and long-term radiation hazard. In addition to current oxide-based fuel-rod designs, the hydride fuel with liquid metal thermal bonding of the fuel-cladding gap is covered. Finally, two of the most promising Generation IV reactor concepts, the Very High Temperature Reactor and the Sodium Fast Reactor, and the accompanying reprocessing technologies, aqueous-based UREX and pyrometallurgical, are summarized. In all of the topics covered, the thermodynamics involved in the material's behavior under irradiation and in the reprocessing schemes are emphasized.

  15. [ISRAEL NEONATOLOGY: PRESENT AND FUTURE].

    Science.gov (United States)

    Dollberg, Shaul

    2016-01-01

    The practice of neonatology in Israel debuted in the 1970s as local enterprises by individual hospitals that needed to provide sick and preterm newly born infants with up-to-date and effective care. Descriptions of research and advances in humane and gentle treatment during neonatal care for preterm infants and their families, as well as prevention of neonatal infections, follow-up of preterm infants and care of full-term infants are presented in this issue. The Israel National Very Low Birth Weight (VLBW) Infant database provides an excellent source of knowledge, which has led to multiple scientific publications. Recent international comparisons of the outcome of preterm VLBW infants, made possible by this unique database in Israel, has provided the neonatal community and the Ministry of Health with insights as to the differences in prognosis between Israel and other countries, especially among extremely low birth weight infants. At the border of viability, mortality in Israelis significantly higher than that reported in other countries and proactive steps undertaken to examine these differences and prompt correctional action should be pursued. The Israel Ministry of Health started positive initiatives and should ensure that their steps are implemented at the preterm infant's bedside.

  16. Source rock hydrocarbons. Present status

    International Nuclear Information System (INIS)

    Vially, R.; Maisonnier, G.; Rouaud, T.

    2013-01-01

    This report first presents the characteristics of conventional oil and gas system, and the classification of liquid and gaseous non conventional hydrocarbons, with the peculiar case of coal-bed methane. The authors then describe how source rock hydrocarbons are produced: production of shale oils and gases (horizontal drilling, hydraulic fracturing, exploitation) and of coal-bed methane and coal mine methane. In the next part, they address and discuss the environmental impact of source rock hydrocarbon production: installation footprint, water resource management, drilling fluids, fracturing fluids composition, toxicity and recycling, air pollution, induced seismicity, pollutions from other exploitation and production activities. They propose an overview of the exploitation and production of source rock gas, coal-bed gas and other non conventional gases in the world. They describe the current development and discuss their economic impacts: world oil context and trends in the USA, in Canada and other countries, impacts on the North American market, on the world oil industry, on refining industries, on the world oil balance. They analyse the economic impacts of non conventional gases: development potential, stakes for the world gas trade, consequence for gas prices, development opportunities for oil companies and for the transport sector, impact on CO 2 emissions, macro-economic impact in the case of the USA

  17. Radiation epidemiology: Past and present

    International Nuclear Information System (INIS)

    Boice, J.D. Jr.

    1997-01-01

    Major advancements in radiation epidemiology have occurred during the last several years in studies of atomic bomb survivors, patients given medical radiation, and radiation workers, including underground miners. Risks associated with the Chernobyl accident, indoor radon and childhood exposure to I-131 have yet to be elucidated. Situations in the former Soviet Union around Chelyabinsk, a nuclear installation in the southern Urals, and in the Altai, which received radioactive fallout from weapons testing at Semipalatinsk, Kazakhstan, have the potential to provide information on the effects of chronic radiation exposure. Since Roentgen's discovery of x-rays just 100 years ago, a tremendous amount of knowledge has been accumulated about human health effects following irradiation. The 1994 UNSCEAR report contains the latest compilation and synthesis of radiation epidemiology. This overview will cover epidemiology from a radiation perspective. The different types of study methodologies will be described, followed by a kaleidoscope coverage of past and present studies; ending with some remaining questions in radiation epidemiology. This should set the stage for future chapters, and stimulate thinking about implications of the new data on radiation cancer risks

  18. Extraskeletal presentation of Ewing's Sarcoma.

    Science.gov (United States)

    Mangual, Danny; Bisbal-Matos, Luis A; Jiménez-Lee, Ricardo; Vélez, Román; Noy, Miguel

    2018-03-01

    The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma. Chemotherapy included 8 cycles of doxorubicin, vincristine, and cyclophosphamide, with alternating cycles of etoposide and ifosfamide. A year later, a second wide excision of the mass was performed, followed by bilaminate skin substitute and skin graft placement for reconstruction of the soft-tissue defect. After chemotherapy, a follow-up PET scan showed no signs of re-uptake in any soft tissue or skeletal structures. After 2 years, the patient remains in complete remission.

  19. Clinical presentation of primary hypothyroidism

    International Nuclear Information System (INIS)

    Ahmed, B.; Hussain, T.; Memon, A. R.; Solangi, G. A.

    2001-01-01

    Objective: To determine the clinical preparation of primary hypothyroidism at the time of diagnosis. Design: It was an observational and prospective study. Place and Duration of Study: Civil Hospital, Karachi from 1st January 1997 to 31st December 1997. Subjects and Methods: Forty-eight consecutive cases of primary hypothyroidism were included. A detailed history with special emphasis on symptoms and signs was conducted and the findings observed were noted in previously made proforma. Results: Primary hypothyroidism was found to be 5 times more common in female patients, with male to female ratio 1:5, majority of cases (33.33%) were between 41 to 50 years of age. The common symptoms at the time of presentation were tiredness (95.8%), weakness (91.6%), weight gain (85.4%), hoarseness of voice (83.3%), cold intolerance (77.0%) and constipation (75.0%) in patients. Physical signs observed were delayed relaxation of ankle jerk (93.7%), periorbital edema (83.3%), thick tongue (62.5%), goiter (50.0%), dry and coarse skin (47.9%) in patients. Serum, TSH, T4 and T3 were performed in all cases through immunoradiometric technique. Serum TSH was markedly elevated, with normal to reduced T4 and T3 levels in all cases. Conclusion: Primary hypothyroidism was found more common in female than male of the age group 41-50 years. Weakness, tiredness, hoarseness of voice and constipation were the common symptoms noted. The diagnosis is almost certain on clinical grounds if the patient happens to have delayed relaxation of ankle jerks and periorbital puffiness. (author)

  20. Past and present Aral Sea

    Science.gov (United States)

    Dukhovniy, Viktor; Stulina, Galina; Eshchanov, Odylbek

    2013-04-01

    The tragedy of disappearing of Aral Sea is well known to the World. Before and after collapse of Soviet Union, a huge quantity of scientific and popular editions described with grief the situation around the Aral Sea. After the NIS states became independent, World Bank, UNDP, UNEP in proper competition with each other had provided some assessment of the situation through presentation of some small and medium grants, but after 2000, the local population remained alone with own problems. Although on the eyes of the present generation a unique transformation of great water body into deserts took place, the global scientific community did not find forces and financing for real and detail investigation of the processes accompanying the Sea shrinking and land formation. We should acknowledge and give big respect to NATO, later to German Government that through GTZ (now GIZ) - German International Collaboration Agency - and GFZ (Potzdam) paid attention to this area of environment crisis and organized scientific and protective design in the so-called Priaralie - the territory around the drying Sea and delta of the two rivers - Amudarya and Syrdarya. Thank to this assistance, the local specialists in collaboration with limited a number of foreign scientists (N.Aladin, P.Zavialov, Joop de Schutter, Hans Wilps, Hedi Oberhansli) organized significant works for detail socioeconomic, ecological and hydrological assessment situation in Priaralie and on the Aral sea coast. On this base, Ministry of Agriculture and Water resources of Uzbekistan and State Committee of Water resources of Kazakhstan developed a plan of rehabilitation of Amudarya and Syrdarya deltas and started implementation of these projects. If Kazakh water authority moved ahead in wetland restoration faster, a forestation of delta and drying bed of Aral Sea got big success in Uzbek territory. 244 thousands hectares of saxsaul and tamarix were planted for protection of the Priaralie. By request of GTZ SIC, ICWC

  1. Como responder ao momento presente?

    Directory of Open Access Journals (Sweden)

    Maria Filomena Molder

    2013-12-01

    Full Text Available http://dx.doi.org/10.5007/1984-784X.2013v13n19p13 Foi com esta pergunta — já um efeito de um primeiro encontro entre Irene Pimentel e eu própria — que decidimos desafiar colegas, estudantes e funci­onários da nossa Faculdade, FCSH (Faculdade de Ciências Sociais e Huma­nas, de outras Faculdades da Universidade Nova de Lisboa, de outras Uni­versidades e todos os interessados em con­siderar e discutir em comum aquilo que se passava em Portugal e que no anúncio da Jornada de 6 de De­zembro de 2012 se descrevia como um “processo de desmantela­mento social, económico e cultural sem precedentes — pese embora tantas compara­ções, baseadas na premissa da ‘eterna repetição’ — e cujas consequências não param de exceder as previsões dos responsáveis por esse desmantelamento”. Acedendo com todo o empenho e gratidão ao convite que me foi dirigido por Humberto Brito para fazer uma resenha da Jornada a publicar no primeiro número de Forma de Vida (saúdo a revista e o título, decidi-me, no entanto, a pôr de lado a resenha, que sob a forma de “Editorial” será em breve publi­cada no blogue Responder ao Momento Presente, entre­tanto criado, conjuntamente com os textos escritos pelos nossos convidados, com as parti­cipações de pessoas que corresponderam ao nosso apelo e ainda com contri­bui­ções que se alargaram para lá da Jornada; a que se juntará uma gravação em video, também disponível no Youtube.   Texto publicado originalmente em Forma de Vida, Lisboa, n.1, fev. 2013. Agrade­cemos à autora por permitir a republicação neste número do Boletim. [N.E.

  2. Draft Workshop Summary and Presentations

    International Nuclear Information System (INIS)

    Houssin, Didier; ); Tam, Cecilia; ); Cameron, Ron; ); Paillere, Henri; ); Lisann, Beth; Ehlert, Andreas; Kaufer, Barry; Decobert, Veronique; Beutier, Didier; Crepin, Anne; Aurela, Jorma; Baritaud, Manuel; ); Rooke, Oliver; Blundell, Neil; ); Shropshire, David; Noren, Bjarne; Blundell, Neil; ); Dujardin, Thierry; )

    2014-01-01

    the electricity market reform in the UK); Session 4: Nuclear regulation and safety (Regulation for new build and new comer countries and Fukushima Safety Action Plan; WANO's contribution to strengthen nuclear safety worldwide; Strengthening Good Practice within Nuclear Safety post Fukushima-Daiichi); Session 5: Key Message for the Road-map update (Outline current status of nuclear technology development and need for additional R and D to address increased safety requirements; Provide an updated vision for nuclear energy's role in a low carbon energy system; Identify barriers and actions needed to accelerate the development and deployment of nuclear technologies to meet the Road-map vision; Identify and share lessons learned and best practices in nuclear regulation, front and back end fuel cycle practices, construction and financing. This document brings together the available presentations (slides) given at this workshop: 1 - Road-map overview and role of nuclear in IEA scenarios (Cecilia Tam, IEA); 2 - The Direction Of The 2014 Nuclear Road-map (Ron Cameron, OECD/NEA); 3 - Technology development needs for nuclear energy, 1.a: Reactor technology (Henri Paillere, OECD/NEA); 4 - IEA/NEA Nuclear Technology Road-map Update (Beth Lisann, US-DoE); 5 - Update of the Nuclear Energy Technology Road-map - Nuclear Decommissioning (Andreas Ehlert, E-On); 6 - Overcoming barriers to nuclear build - other than financing (Veronique Decobert, Westinghouse); 7 - Development of the supply chain: relying on local industrial involvement (Didier Beutier, Areva); 8 - Training and capacity development needs (Jorma Aurela, Finnish Ministry of Employment and the Economy); 9 - Key conditions for successful Nuclear new build financing (Anne Crepin, Areva); 10 - Regulation for New Build and Newcomers, IAEA Nuclear Safety Action Plan (David Shropshire, IAEA); 11 - WANO's contribution to strengthen nuclear safety worldwide (Bjarne Noren, WANO); 12 - Strengthening Good Practice within Nuclear Safety

  3. Complete agenesis of the dorsal pancreas: Case report with ...

    African Journals Online (AJOL)

    pancreatic head and uncinate process were normal, but the distal neck, body ... The neck, body, tail, and cephalic aspects of the head of the pancreas originate from the .... Embryology, normal variation, and congenital anomalies of the pancreas. ... M. A 3D reconstruction of pancreas development in the human embryos.

  4. Three-dimensional colour Doppler of ductus venosus agenesis in ...

    African Journals Online (AJOL)

    was bilateral pleural effusion with generalised subcutaneous oedema suggestive of hydrops fetalis (Fig. 1B). Colour Doppler sonography revealed the umbilical vein draining into the portal sinus with non-visualisation of the DV, which would connect the portal sinus with the inferior vena cava (IVC) (Figs 1C and 1D). Hence ...

  5. Laparoscopic Colectomy for a Patient with Congenital Renal Agenesis

    African Journals Online (AJOL)

    The right colon was mobilized by lateral‑to‑medial extension of a retroperitoneal dissection between the fusion fascia and the anterior renal fascia. The right testicular vessels were preserved without injury to the anterior renal fascia; however, the right ureter could not be detected. The operation was performed safely. Thus ...

  6. PAX9 gene mutations and tooth agenesis: A review

    Czech Academy of Sciences Publication Activity Database

    Bonczek, Ondřej; Balcar, V. J.; Šerý, Omar

    2017-01-01

    Roč. 92, č. 5 (2017), s. 467-476 ISSN 0009-9163 Institutional support: RVO:67985904 Keywords : PAX9 * gene * hypodontia Subject RIV: FF - HEENT, Dentistry OBOR OECD: Dentistry, oral surgery and medicine Impact factor: 3.326, year: 2016

  7. Laparoscopic Colectomy for a Patient with Congenital Renal Agenesis

    African Journals Online (AJOL)

    2013-10-07

    the tumor could not be elevated by injection of saline into ... on Monday, October 07, 2013, IP: 41.135.175.93] || Click here to download free Android application for this journal .... the ureter facilitate the safe treatment of patients with congenital ...

  8. [Dental agenesis. Results of a prospective study of 30 cases].

    Science.gov (United States)

    Romieux, G; Bonin, B; Laure, B; Van Hove, A; Goga, D

    2000-11-01

    We conducted a prospective study to determine the types of bone anomalies observed in different types of dental agenesia. This prospective series included 30 patients who attended our pluridisciplinary clinic since 1988. There were 22 cases of non syndromal agenesia and 8 cases of syndromal agenesia. Patients consulted for a variety of reasons, no specific sign was found. The diagnosis was based on the panoramic x-ray and confirmed at the genetic consultation. Results were systemized by localization of the agenesia. In all cases, the height of the bone crest was preserved compared with the adjacent teeth. In the anterior part of the maxillary, the bone crest was thin showing a water drop aspect. In the posterior maxillary, there was a decrease in the subsinusal height due to invagination of the floor of the sinus. In the anterior part of the mandible, the crest had a knife blade aspect but no loss of height and in the posterior part, a preserved distance between the residual crest rim and the dental canal. In 60% of the solitary agenesias in the anterior part of the maxillary, augmentation was not required. A sinus graft was required in all cases involving the posterior maxillary. Multiple anterior or lateral agenesias were treated with a parietal graft.

  9. The role of color and power Doppler ultrasound in the prenatal diagnosis of sirenomelia.

    Science.gov (United States)

    Patel, S; Suchet, I

    2004-11-01

    Sirenomelia is a rare congenital abnormality characterized by a variety of anomalies involving the lower limbs, severe oligohydramnios, bilateral renal agenesis, anorectal atresia and aberrant fetal vasculature. The prenatal diagnosis and differentiation from isolated bilateral renal agenesis is severely limited by the accompanying oligohydramnios that hinders visualization of fetal anatomy. We present three prospectively assessed cases of sirenomelia, two of which had bilateral renal agenesis, and all of which had a single umbilical artery derived from the aberrant vasculature that accompanies the syndrome.

  10. Controlled thermonuclear fusion. Present state and prospective

    International Nuclear Information System (INIS)

    Consoli, T.

    1976-01-01

    The interest of thermonuclear fusion for energy production is underlined. The present state of the research in this field is presented, emphasis being given to Tokamak configurations. The problems concerning confinement and additional heating in these devices are presented [fr

  11. Ten steps to successful poster presentation.

    Science.gov (United States)

    Hardicre, Jayne; Devitt, Patric; Coad, Jane

    Receiving a letter confirming acceptance for you to present a poster at a conference can evoke mixed emotions. Joy, panic, fear and dread are among the many possible emotions and this is not exclusive to first time presenters. Developing an effective poster presentation is a skill that you can learn and can provide a rewarding way to present your work in a manner less intimidating than oral presentation (Shelledy, 2004). The key to successful poster presentation is meticulous, timely, well informed preparation. This article outlines ten steps to help guide you through the process to maximize your success.

  12. 2016 Annual Technology Baseline (ATB) - Webinar Presentation

    Energy Technology Data Exchange (ETDEWEB)

    Cole, Wesley; Kurup, Parthiv; Hand, Maureen; Feldman, David; Sigrin, Benjamin; Lantz, Eric; Stehly, Tyler; Augustine, Chad; Turchi, Craig; Porro, Gian; O' Connor, Patrick; Waldoch, Connor

    2016-09-13

    This deck was presented for the 2016 Annual Technology Baseline Webinar. The presentation describes the Annual Technology Baseline, which is a compilation of current and future cost and performance data for electricity generation technologies.

  13. Present studies on the radiosterilized drugs

    International Nuclear Information System (INIS)

    Marciniec, B.; Dettlaff, K.

    2007-01-01

    Lecture presents present status of radiosterilization of drugs and medical materials as compared to other sterilization methods. Literature review is shown on degradation of different kinds of drugs sterilized upon action of the sterilising doses of radiation

  14. Climatic Change--Past, Present & Future

    Science.gov (United States)

    Lindholm, Roy C.

    1976-01-01

    Presented is a review of studies investigating factors affecting climatic changes in the Earth's atmosphere--past, present, and future. Dating methods, particularly the Oxygen 18/16 method, are discussed. (SL)

  15. Unexpected effects of computer presented procedures

    International Nuclear Information System (INIS)

    Blackman, H.S.; Nelson, W.R.

    1988-01-01

    Results from experiments conducted at the Idaho National Engineering Laboratory have been presented regarding the computer presentation of procedural information. The results come from the experimental evaluation of an expert system which presented procedural instructions to be performed by a nuclear power plant operator. Lessons learned and implications from the study are discussed as well as design issues that should be considered to avoid some of the pitfalls in computer presented or selected procedures

  16. Reflections: Improving Medical Students' Presentation Skills.

    Science.gov (United States)

    Tarkowski, Radoslaw

    2017-12-01

    Both good communication and presentation skills on the part of an academic teacher are crucial when trying to generate students' interest in the subject of a lecture. More generally, our task is to share knowledge in the most effective way possible. It is also worth teaching students presentation skills, as today's students are tomorrow's teachers. An engaging presentation is a powerful tool. There are some rules for presenting which I consider worthy of being discussed and taught at a medical university.

  17. Unexpected effects of computer presented procedures

    International Nuclear Information System (INIS)

    Blackman, H.S.; Nelson, W.R.

    1988-01-01

    Results from experiments conducted at the Idaho National Engineering Laboratory will be presented regarding the computer presentation of procedural information. The results come from the experimental evaluation of an expert system which presented procedural instructions to be performed by a nuclear power plant operator. Lessons learned and implications from the study will be discussed as well as design issues that should be considered to avoid some of the pitfalls in computer presented or selected procedures. 1 ref., 1 fig

  18. Case report

    African Journals Online (AJOL)

    abp

    27;70(4):391-8. PubMed | Google Scholar. 5. Nazaroglu H, Mete A, Bukte Y, Smsek M. Agenesis of the right lung presenting as a pulmonary infection. Clin Radiol. 2002;. 57(6): 529-30. PubMed | Google Scholar. 6. Koseoglua N, Ucana ES, Cavdarb C. Right lung agenesis and left lung bronchiectasis. Resp Med Extra. 2005 ...

  19. Intuitionistic Assessment Of Behavioural Present Value*

    OpenAIRE

    Piasecki Krzysztof

    2014-01-01

    The article discussesd the impact of chosen behavioural factors on the imprecision of present value assessment. The formal model of behavioural present value is offered as a result of this discussion. The behavioural present value is described here as an intuitionistic fuzzy set. The significance of the replacement of a fuzzy set by an intuitionistic fuzzy set is proved.

  20. The Design Space of Information Presentation

    DEFF Research Database (Denmark)

    May, Michael; Petersen, Johannes

    2007-01-01

    A semiotic approach to the design space of information presentation is presented in which Formal Concept Analysis (FCA) is used to represent and explore attributes of abstract sign types and the media (graphical, haptic, acoustic, gestic) through which they are presented as specific...

  1. The Statue of Liberty Deed of Presentation.

    Science.gov (United States)

    Schamel, Wynell Burroughs; Blondo, Richard A.

    1992-01-01

    Presents a history lesson based on the deed of presentation for the Statue of Liberty. Discusses the conception and history of the statue and its presentation to the United States as a gift from the people of France. Includes a copy of the document, topics for class discussion, and research, writing, and design activities. (DK)

  2. Twelve Tips for Effective Electronic Presentation.

    Science.gov (United States)

    Crosby, Joy

    1994-01-01

    Offers 12 tips for effective electronic presentation. This article is intended for readers who may be considering using electronic presentation for the first time. Offers reasons for its popularity and occasions when it may be used. The tips offer assistance in the design and presentation of electronic material. (LZ)

  3. Intracranial Castleman's disease presenting as hypopituitarism

    International Nuclear Information System (INIS)

    Ribeiro, L.T.; Simao, G.N.; Matos, A.L.M.; Santos, Antonio Carlos; Carlotti, C.G. Jr.; Colli, B.O.; Neder, L.; Ribeiro-Silva, A.; Castro, M. de; Rego, E.

    2004-01-01

    Castleman's disease is an atypical lymphoproliferative disorder that may present as a localized or multicentric form. The involvement of the central nervous system is rare. We describe here a case of Castleman's disease with involvement of the hypothalamus and meninges, presenting as hypopituitarism. Radiological and clinical pathological features are emphasized and a review of the literature is presented. (orig.)

  4. Automatic presentation generation for scholarly hypermedia

    NARCIS (Netherlands)

    S. Bocconi

    2003-01-01

    textabstractAutomatic hypermedia presentation generation uses an information source semantic network first to select the content and then to compose it in the presentation so that the semantic relations between the information items are conveyed to the user. A hypermedia presentation can be

  5. Clinical presentation of familial exudative vitreoretinopathy.

    Science.gov (United States)

    Ranchod, Tushar M; Ho, Lawrence Y; Drenser, Kimberly A; Capone, Antonio; Trese, Michael T

    2011-10-01

    To describe the clinical characteristics, staging and presentation of patients with familial exudative vitreoretinopathy (FEVR) in our clinical practice over the last 25 years. Case series, retrospective review. We included 273 eyes of 145 patients. Data collected from charts included gender, gestational age at birth, birthweight, age at presentation, referring diagnosis, family history, prior ocular surgery, and clinical presentation in each eye. Eyes with invasive posterior segment procedures before initial presentation were excluded. Demographics on presentation and clinical staging. Patients were slightly male predominant (57%) with a mean birthweight of 2.80 kg (range, 740 g-4.76 kg), mean gestational age of 37.8 weeks (range, 25-42), and mean age at presentation of almost 6 years (range, presentation, gestational age, and birthweight. Although a positive family history on presentation may support the diagnosis of FEVR, a negative family history is of little help. The majority of retinal folds extended radially in the temporal quadrants, but radial folds were seen in almost all quadrants. Fellow eyes demonstrated a wide variation in symmetry. The presentation of FEVR may mimic the presentation of other pediatric and adult vitreoretinal disorders, and careful examination is often crucial in making the diagnosis of FEVR. The authors have no proprietary or commercial interest in any of the materials discussed in this article. Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

  6. Antigen Cross-Presentation of Immune Complexes

    Science.gov (United States)

    Platzer, Barbara; Stout, Madeleine; Fiebiger, Edda

    2014-01-01

    The ability of dendritic cells (DCs) to cross-present tumor antigens has long been a focus of interest to physicians, as well as basic scientists, that aim to establish efficient cell-based cancer immune therapy. A prerequisite for exploiting this pathway for therapeutic purposes is a better understanding of the mechanisms that underlie the induction of tumor-specific cytotoxic T-lymphocyte (CTL) responses when initiated by DCs via cross-presentation. The ability of humans DC to perform cross-presentation is of utmost interest, as this cell type is a main target for cell-based immunotherapy in humans. The outcome of a cross-presentation event is guided by the nature of the antigen, the form of antigen uptake, and the subpopulation of DCs that performs presentation. Generally, CD8α+ DCs are considered to be the most potent cross-presenting DCs. This paradigm, however, only applies to soluble antigens. During adaptive immune responses, immune complexes form when antibodies interact with their specific epitopes on soluble antigens. Immunoglobulin G (IgG) immune complexes target Fc-gamma receptors on DCs to shuttle exogenous antigens efficiently into the cross-presentation pathway. This receptor-mediated cross-presentation pathway is a well-described route for the induction of strong CD8+ T cell responses. IgG-mediated cross-presentation is intriguing because it permits the CD8− DCs, which are commonly considered to be weak cross-presenters, to efficiently cross-present. Engaging multiple DC subtypes for cross-presentation might be a superior strategy to boost CTL responses in vivo. We here summarize our current understanding of how DCs use IgG-complexed antigens for the efficient induction of CTL responses. Because of its importance for human cell therapy, we also review the recent advances in the characterization of cross-presentation properties of human DC subsets. PMID:24744762

  7. Poster presentations: valuing all forms of evidence.

    Science.gov (United States)

    Halligan, Phil

    2008-01-01

    This paper aims to assist readers who are considering submitting a poster presentation to a conference to appreciate their true worth and demonstrate the important contribution they can make to nursing scholarship and secondly, to provide new insights to conference reviewers who have the task of selecting appropriate contributions for conferences. The author will argue that the use of poster presentations is an ideal opportunity to disseminate research findings and clinical innovations. It offers a new, interesting, and thought-provoking perspective on poster presentations, which includes appraisal of extant literature and reflections about the significance of poster presentations to nursing scholarship. It highlights the benefits of submitting a poster presentation and concludes with some key points for conference reviewers to consider when deciding to select the suitability of abstracts for poster presentations or concurrent sessions.

  8. Clinical presentation of adult coeliac disease.

    LENUS (Irish Health Repository)

    Tajuddin, T

    2012-02-01

    The mode of presentation of coeliac disease has been changing to more atypical or silent disease. Few studies described the clinical presentation of adult coeliac disease in Ireland in recent years. We retrospectively collected the clinical data for all patients who had a diagnosis of coeliac disease made in our centre between January 07 and December 08. Forty seven adults, predominantly females (n = 30), had a confirmed diagnosis of coeliac disease made during the study period. In our patient cohort, the presenting symptom was diarrhoea in 19 (40%) patients, while 16 patients (34%) did not have any G.I. symptoms, 10 (21%) presented with anaemia. Females presented at a significantly younger age compared to males, with median ages at diagnosis of 44.5 and 57 years, respectively (p = 0.04). Females also presented more commonly with non G.I. symptoms (p = 0.07). The reasons behind this gender difference need further study.

  9. Mediated Teaching for Information Presenting and Understanding

    Directory of Open Access Journals (Sweden)

    Konstantinas Danaitis

    2011-04-01

    Full Text Available Nowadays media is readily moving into various spheres of life. The computer, projector and screen replace trivial chalk and board. Slides are used in various designated areas: promotional presentations at meetings as a form of communication between participants and also as visual presentation of lecture material content of the report. It was found that a human catches up and remembers only about 10% of what he has read, 20% of what he has heard and even 50% of what he heard and saw. Therefore, high-quality and Professional presentation of information in the slide form has a significant impact on the uptake. The authors present a solution how to present the study material qualitatively and use the media system effectively for teaching computer graphics course. This course is taught for VGTU first or second year students of almost all specialities. This presentation of study materials facilitates the teacher's work and improves the readability and solutions of study materials.

  10. Promoting professional development through poster presentations.

    Science.gov (United States)

    Durkin, Gregory

    2011-01-01

    Poster presentations are commonplace at regional and national nursing conferences, although the development of a poster remains an intimidating task for many staff nurses. The author describes the staff development department's role in implementing in-house poster presentation sessions. Nursing staff are provided support and assistance in presenting posters to their colleagues at yearly sessions. The result has been increased comfort and experience with poster creation, participation in professional development activities, and dissemination of nursing practice innovation.

  11. Radiocarbon calibration - past, present and future

    Energy Technology Data Exchange (ETDEWEB)

    Plicht, J. van der E-mail: plicht@phys.rug.nl

    2004-08-01

    Calibration of the Radiocarbon timescale is traditionally based on tree-rings dated by dendrochronology. At present, the tree-ring curve dates back to about 9900 BC. Beyond this limit, marine datasets extend the present calibration curve INTCAL98 to about 15 600 years ago. Since 1998, a wealth of AMS measurements became available, covering the complete {sup 14}C dating range. No calibration curve can presently be recommended for the older part of the dating range until discrepancies are resolved.

  12. Radiocarbon calibration - past, present and future

    International Nuclear Information System (INIS)

    Plicht, J. van der

    2004-01-01

    Calibration of the Radiocarbon timescale is traditionally based on tree-rings dated by dendrochronology. At present, the tree-ring curve dates back to about 9900 BC. Beyond this limit, marine datasets extend the present calibration curve INTCAL98 to about 15 600 years ago. Since 1998, a wealth of AMS measurements became available, covering the complete 14 C dating range. No calibration curve can presently be recommended for the older part of the dating range until discrepancies are resolved

  13. Nasopharyngeal bursitis: from embryology to clinical presentation

    OpenAIRE

    El-Shazly, Amr

    2010-01-01

    AE El-Shazly, S Barriat, PP LefebvreDepartment of Otorhinolaryngology and Head and Neck Surgery, Liege University Hospital, Liege, BelgiumAbstract: Nasopharyngeal bursitis is a relatively rare syndrome characterized by a collection of symptoms that multidisciplinary specialists should be aware of. Here we present an audit of cases presenting to a rhinology clinic over a two-year period, as well as an overview of the relevant embryology and different clinical presentations of nasopharyngeal bu...

  14. Subacute sclerosing panencephalitis presenting as mania

    Directory of Open Access Journals (Sweden)

    Aggarwal Ashish

    2011-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a rare, invariably fatal degenerative disease of the central nervous system developing after measles infection. Besides neurological symptoms as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. We here report a case of 14 year old male who initially presented with manic symptoms and then subsequently diagnosed to be suffering from SSPE. Improtance of ruling our organic conditions is emphasized.

  15. Trismus: An unusual presentation following road accident

    Directory of Open Access Journals (Sweden)

    Thakur Jagdeep

    2007-01-01

    Full Text Available Trismus due to trauma usually follows road accidents leading to massive faciomaxillary injury. In the literature there is no report of a foreign body causing trismus following a road accident, this rare case is an exception. We present a case of isolated presentation of trismus following a road accident. This case report stresses on the thorough evaluation of patients presenting with trismus following a road accident.

  16. Pulmonary strongyloidiasis. Presentation of two cases

    International Nuclear Information System (INIS)

    Munive, Abraham Ali; Torres D, Carlos A Lasso A Javier J; Ojeda Leon, Paulina; Acosta R, Nohora

    2002-01-01

    We describe two case reports of immune-suppressed patients receiving oral steroids and who presented lung Strongyloidiasis; later they evolved toward respiratory failure, with different clinical courses. One developed severe hypoxaemia, hemodynamic instability and death. The worst prognosis in this patient was determined by diffuse infiltrates and the resulting lung injury. The other one presented a stable clinical course and evolved to full recovery; this case presented a cavern in the chest X-ray that could represent a preliminary phase for lung extension of the infection. The difference in the evolution of these two patients is determined by the different presentation of the lung damage

  17. Visual communication in presentation on physics

    Science.gov (United States)

    Grebenyuk, Konstantin A.

    2005-06-01

    It is essential that our audience be attentive during lecture, report or another presentation on physics. Therefore we have to take care of both speech and visual communication with audience. Three important aspects of successful visual aids use are singled out in this paper. The main idea is that physicists could appreciably increase efficiency of their presentations by use of these simple principles of presentation art. Recommendations offered are results of special literature research, author' s observations and experience of communication with skilled masters of presentations.

  18. A rare presentation of hydatid cyst

    International Nuclear Information System (INIS)

    Sabir, N.; Yildirim, B.; Alatas, E.; Cetin, B.

    2005-01-01

    Hydatid disease, although known to occur in most body areas, is extremely rare in the female reproductive system. There are different modes of presentation for the disease; however, we report and discuss a case presented with cystic vesicles passing through the vagina, which is considered as a rare presentation for secondary involvement of the uterus and both ovaries. We confirmed diagnosis with radiological examinations and serological tests. We operated on the patient, and studied the excised cysts microscopically. The gynecologist should be aware of hydatid cyst when vaginally passing a grape like vesicle is presented by the patient. (author)

  19. Biased lineups: sequential presentation reduces the problem.

    Science.gov (United States)

    Lindsay, R C; Lea, J A; Nosworthy, G J; Fulford, J A; Hector, J; LeVan, V; Seabrook, C

    1991-12-01

    Biased lineups have been shown to increase significantly false, but not correct, identification rates (Lindsay, Wallbridge, & Drennan, 1987; Lindsay & Wells, 1980; Malpass & Devine, 1981). Lindsay and Wells (1985) found that sequential lineup presentation reduced false identification rates, presumably by reducing reliance on relative judgment processes. Five staged-crime experiments were conducted to examine the effect of lineup biases and sequential presentation on eyewitness recognition accuracy. Sequential lineup presentation significantly reduced false identification rates from fair lineups as well as from lineups biased with regard to foil similarity, instructions, or witness attire, and from lineups biased in all of these ways. The results support recommendations that police present lineups sequentially.

  20. Selected Regional Judicial Officer Cases, 2005 - Present

    Data.gov (United States)

    U.S. Environmental Protection Agency — This dataset contains selected cases involving EPA's Regional Judicial Officers (RJOs) from 2005 to present. EPA's Regional Judicial Officers (RJOs) perform...

  1. Poster Presentations: Conceptualizing, Constructing and Critiquing

    Science.gov (United States)

    Newbrey, Michael G.; Baltezore, Joan M.

    2006-01-01

    Posters are commonly used as tools for disseminating information at scientific meetings, but many students lack an understanding of "good" poster characteristics. We present a set of characteristics for use in constructing posters for scientific meetings and classroom presentations along with tips on critiquing posters to enhance their…

  2. Applying Technology To Facilitate Poster Presentations.

    Science.gov (United States)

    Marek, Pam; Christopher, Andrew N.; Koenig, Cynthia S.

    2002-01-01

    Promotes the use of presentation software in psychology courses to teach students technological skills that prepare them for the future. Explains that many graduates in psychology are employed in other fields after graduation. Discusses the use of presentation software with a focus on poster preparation using Microsoft PowerPoint software. (CMK)

  3. Speechline - a method for teaching oral presentation

    DEFF Research Database (Denmark)

    Juel, Henrik

    2015-01-01

    The Speech-line method aims at improving the active rhetorical skills of students, i.e. their ability to present academic subjects in a clear and convincing manner. This is relevant not only for everyday classroom work, but also for oral exams, for presenting research projects, and for addressing...

  4. Becker muscular dystrophy: an unusual presentation.

    OpenAIRE

    Thakker, P B; Sharma, A

    1993-01-01

    A 15 year old boy who presented with passing painless dark urine was found to have myoglobinuria. His creatine phosphokinase was raised, and a muscle biopsy specimen showed non-specific dystrophic changes. Subsequent DNA analysis led to the diagnosis of Becker muscular dystrophy. Myoglobinuria may be a presenting symptom of Becker muscular dystrophy.

  5. Film sound in preservation and presentation

    NARCIS (Netherlands)

    Campanini, S.

    2014-01-01

    What is the nature of film sound? How does it change through time? How can film sound be conceptually defined? To address these issues, this work assumes the perspective of film preservation and presentation practices, describing the preservation of early sound systems, as well as the presentation

  6. The spanish radiotherapy park: past and present

    International Nuclear Information System (INIS)

    Tormo Ferrero, Manuel J.

    2001-01-01

    The present article has as objective to provide a general overview on the spanish radiotherapy park, presenting how was its start and evolution until the current state. Considering only the units of teletherapy and the accelerators. Actually in Spain there is 28 units of Cobalt therapy, in functioning during the last two decades, being advised a rapid substitution to accelerators

  7. Dynamic bowel obstruction: aetiology, clinical presentation ...

    African Journals Online (AJOL)

    This study was conducted to describe in our region, the aetiology, clinical presentation, management and outcome of dynamic bowel obstruction. Data were analyzed using SPSS software system. A total of 342 patients were studied. Males outnumbered females by a ratio of 2.1: 1. The median age of patients at presentation ...

  8. Acute intermittent porphyria presenting as progressive muscular ...

    African Journals Online (AJOL)

    Acute intermittent porphyria, the most common porphyria affecting the nervous system, typically presents with neurovisceral crises followed by a motor neuropathy. We describe a 23-year-old black South African man presenting with a progressive stuttering, lower motor neuron syndrome developing over months. He had not ...

  9. Story Presentation Effects on Children's Retell Content

    Science.gov (United States)

    Schneider, Phyllis; Dube, Rita Vis

    2005-01-01

    This study investigated the possibility that the amount of content children include in their stories is affected by how stories are presented. Simple stories were presented to kindergarten and Grade 2 children in 3 conditions: orally (oral only), pictorially (pictures only), and combined oral and pictures. The kindergarteners recalled more content…

  10. Normothermic thyroid storm: an unusual presentation

    Science.gov (United States)

    Sabir, Anas Ahmad; Sada, Kabiru; Yusuf, Bashir O.; Aliyu, Idris

    2016-01-01

    Thyroid storm is a rare life-threatening emergency due to thyrotoxicosis. A 30-year-old female presented with restlessness, tachycardia and vomiting but with normothermia which is an unusual presentation. There is the need for clinicians to be aware of atypical clinical features that can make the diagnosis of thyroid storm difficult. PMID:27540465

  11. NUT carcinoma presenting in the palate

    DEFF Research Database (Denmark)

    Bjornstrup, Libana Raffoul; Reibel, Jesper; Kiss, Katalin

    2017-01-01

    NUT carcinomas (NC) are rare and aggressive tumours characterized by chromosomal rearrangements of the gene encoding for nuclear protein of the testis (NUT) located on chromosome 15q14. This article presents a case of a 60-year-old woman diagnosed with NC presenting as a fast growing primary tumo...

  12. Central nervous system tuberculomata presenting as internuclear ...

    African Journals Online (AJOL)

    Central nervous system (CNS) tuberculoma can have variable presentation depending upon the site and number of tuberculomata. We are reporting a rare case of a 15 years old girl who presented to our hospital with binocular diplopia on right gaze. Clinical examination revealed left sided internuclear ophthalmoplegia ...

  13. WWNPQFT-2010 - Slides of the presentations

    International Nuclear Information System (INIS)

    Fried, H.M.; Huber, M.Q.; Grandou, T.; Bianchi, E.; Gracey, J.; Reys, V.; Jevicki, A.; Ferrante, D.; Bouakaz, K.; Spielmann, D.; Cucchieri, A.; Culetu, H.; Gelis, F.; Zwanziger, D.; Candelpergher, B.; Bender, C.

    2013-01-01

    This document is made up of the slides of the presentations. The object of this workshop is to consolidate and publicize new efforts in non-perturbative field theories. The main topics are quantum chromodynamics, Yang-Mills theory, effective locality, the Gribov-Zwanziger Lagrangian, and renormalization. A presentation is dedicated to the initial stages of high energy nucleus-nucleus collisions

  14. Extranodal presentation in patients with acquired immunodeficiency ...

    African Journals Online (AJOL)

    NHL is the second most common malignancy associated with HIV infection. The clinical presentation varies, and while patients may present with symptomatic lymphadenopathy, many have extranodal disease. Extranodal lymphomas arise from tissue other than lymph nodes and even sites that normally contain no lymphoid ...

  15. Changing Dynamics in the Voluntary Market (Presentation)

    Energy Technology Data Exchange (ETDEWEB)

    Heeter, J.

    2014-12-01

    Voluntary green power markets are those in which consumers and institutions voluntarily purchase renewable energy to match their electricity needs. This presentation, presented at the Renewable Energy Markets Conference in December 2014, outlines the voluntary market in 2013, including community choice aggregation and community solar.

  16. 25 CFR 11.903 - Presenting officer.

    Science.gov (United States)

    2010-04-01

    ... OF INDIAN AFFAIRS, DEPARTMENT OF THE INTERIOR LAW AND ORDER COURTS OF INDIAN OFFENSES AND LAW AND ORDER CODE Children's Court § 11.903 Presenting officer. (a) The agency superintendent and the chief magistrate of the children's court shall jointly appoint a presenting officer to carry out the duties and...

  17. Genitourinary complications as initial presentation of inherited ...

    African Journals Online (AJOL)

    Epidermolysis bullosa (EB) is a rare disorder that presents with urological complications. We present a 6-year-old boy admitted with urological symptoms that revealed an inherited EB misdiagnosed. We also review the literature on this disorder and management of the common urological complications.

  18. Using the Microcomputer for Advertising Research Presentations.

    Science.gov (United States)

    Larkin, Ernest F.

    A midwestern university is testing a program that uses the Apple II computer to help students in an advertising research course develop their skills in preparing and presenting research reports using computer generated graphics for both oral and written presentations. One of the course requirements is the preparation of a final project, including…

  19. Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic ...

    African Journals Online (AJOL)

    Background: Acute Lymphoblastic Leukaemia in children commonly presents with osteo articular manifestations that may mimic Juvenile Idiopathic Arthritis. This may create considerable diagnostic difficulty and lead to delay in commencing appropriate treatment. Case: An eight year old boy who presented with multiple ...

  20. Diffuse benign gastric inflammatory hyperplastic polyps presenting ...

    African Journals Online (AJOL)

    Benign gastric inflammatory hyperplasic polyps are benign lesions that rarely occur in young age. We report a case of diffuse benign gastric inflammatory hyperplastic polyps in a 19 year old boy who presented with cough, nausea, and hematamesis. In the presented case symptoms such as nausea and vomiting are non ...

  1. Processing of visually presented clock times.

    Science.gov (United States)

    Goolkasian, P; Park, D C

    1980-11-01

    The encoding and representation of visually presented clock times was investigated in three experiments utilizing a comparative judgment task. Experiment 1 explored the effects of comparing times presented in different formats (clock face, digit, or word), and Experiment 2 examined angular distance effects created by varying positions of the hands on clock faces. In Experiment 3, encoding and processing differences between clock faces and digitally presented times were directly measured. Same/different reactions to digitally presented times were faster than to times presented on a clock face, and this format effect was found to be a result of differences in processing that occurred after encoding. Angular separation also had a limited effect on processing. The findings are interpreted within the framework of theories that refer to the importance of representational codes. The applicability to the data of Bank's semantic-coding theory, Paivio's dual-coding theory, and the levels-of-processing view of memory are discussed.

  2. Clinical presentation and staging of Hodgkin lymphoma

    DEFF Research Database (Denmark)

    Gallamini, Andrea; Hutchings, Martin; Ramadan, Safaa

    2016-01-01

    , sometimes HL is a subtle disease, difficult to diagnose for the paucity of symptoms, the absence of physical findings, or for concomitant immunologic disorders: a compete overview of the common and rare patterns of HL clinical presentation will be also offered. The future perspective of PET scan use......In the present chapter the authors present a brief overview of the diagnostic methods proposed over time for Hodgkin lymphoma (HL) spread detection, moving from surgical procedures, through standard radiological and functional imaging techniques to the present state of the art for HL staging...... tomography (FDG-PET) scan in the armamentarium of staging procedures will be presented and commented, with a special emphasis on the utility of special procedures, such as bone marrow trephine biopsy, which is deemed no longer needed in the PET era. While the HL diagnosis is straightforward in most cases...

  3. Menkes Disease Presenting with Epilepsia Partialis Continua

    Directory of Open Access Journals (Sweden)

    Tamer Rizk

    2014-01-01

    Full Text Available Aim. We aim to describe a female patient with Menkes disease who presented with epilepsia partialis continua. Case Presentation. Seventeen-months-old Saudi infant was presented with repetitive seizures and was diagnosed to have epilepsia partialis continua. Discussion. Menkes disease (OMIM: 309400 is considered a rare, X-linked recessive neurodegenerative disorder resulting from a mutation in the gene coding for the copper transporting ATPase (ATP7A. Affected individuals usually present with kinky hair, skeletal changes, prolonged jaundice, hypothermia, developmental regression, decreased tone, spasticity, weakness, and therapy resistant seizures. Conclusion. Raising awareness of abnormal presentation of this rare disease may help in the control of seizures through subcutaneous copper supplementation.

  4. Intracellular events regulating cross-presentation

    Directory of Open Access Journals (Sweden)

    Peter eCresswell

    2012-06-01

    Full Text Available Cross-presentation plays a fundamental role in the induction of CD8-T cell immunity. However, although more than three decades have passed since its discovery, surprisingly little is known about the exact mechanisms involved. Here we give an overview of the components involved at different stages of this process. First, antigens must be internalized into the cross-presenting cell. The involvement of different receptors, method of antigen uptake, and nature of the antigen can influence intracellular trafficking and access to the cross-presentation pathway. Once antigens access the endocytic system, different requirements for endosomal/phagosomal processing arise, such as proteolysis and reduction of disulfide bonds. The majority of cross-presented peptides are generated by proteasomal degradation. Therefore, antigens must cross a membrane barrier in a manner analogous to the fate of misfolded proteins in the endoplasmic reticulum (ER that are retrotranslocated into the cytosol for degradation. Indeed, some components of the ER-associated degradation (ERAD machinery have been implicated in cross-presentation. Further complicating the matter, endosomal and phagosomal compartments have been suggested as alternative sites to the ER for loading of peptides on MHC class I molecules. Finally, the antigen presenting cells involved, particularly dendritic cell subsets and their state of maturation, influence the efficiency of cross-presentation.

  5. Information presentation in power plant control rooms

    International Nuclear Information System (INIS)

    Kautto, A.

    1984-11-01

    The objective of this study is to support operators' work especially in the control rooms of power plant. The exemplified process is a pressurized water (nuclear) reactor (PWR). The man-process interface is an information system that covers information refining, information presentation, information system handling, and process control. THe emphasis in this study is on the organization and presentation of information and on the alert function that is part of the information system. Another goal is to design the alert function so as to radically reduce the number of alarms during plant shutdown, e.g. during the refuelling or maintenance period and during a disturbance. Further, the experimental validation of CFMS (Critical Function Monitoring System), developed by Combustion Engineering, Inc. in the U.S.A. is described briefly. The validation was made at the Loviisa training simulator in the autumn of 1982. CFMS is a safety-related functional alarm system. The functional decomposition of information has turned out to be successful and it is helpful in designing displays. Preliminary criteria for designing displays, the structure of the information presentation system and the illustration of main interactions are presented. General practical ideas on designing the alert function seem very promising. Preliminary results of the CFMS validation are presented. Further, some ideas are presented on how to carry out the analysis and how to make such validations in the future. A new idea for the evaluation of core safety is presented, based on control theory concepts

  6. Manipulating affective state using extended picture presentations.

    Science.gov (United States)

    Sutton, S K; Davidson, R J; Donzella, B; Irwin, W; Dottl, D A

    1997-03-01

    Separate, extended series of positive, negative, and neutral pictures were presented to 24 (12 men, 12 women) undergraduates. Each series was presented on a different day, with full counterbalancing of presentation orders. Affective state was measured using (a) orbicularis oculi activity in response to acoustic startle probes during picture presentation, (b) corrugator supercilii activity between and during picture presentation, and (c) changes in self-reports of positive and negative affect. Participants exhibited larger eyeblink reflex magnitudes when viewing negative than when viewing positive pictures. Corrugator activity was also greater during the negative than during the positive picture set, during both picture presentation and the period between pictures. Self-reports of negative affect increased in response to the negative picture set, and self-reports of positive affect were greatest following the positive picture set. These findings suggest that extended picture presentation is an effective method of manipulating affective state and further highlight the utility of startle probe and facial electromyographic measures in providing on-line readouts of affective state.

  7. Transforming your presentation into a publication.

    Science.gov (United States)

    Schrager, Sarina

    2010-04-01

    Publication in scientific journals is required for academic promotion at universities. It is also the method by which most family medicine faculty members disseminate their scholarly work, establish a reputation in their field, and develop a clinical, teaching, or scholarly expertise. Publications are able to reach a broader audience than are presentations, which makes them the epitome of academic scholarship. Most academic family physicians give presentations to students, residents, or colleagues as part of their regular job duties and at regional and national meetings, but few write articles based on those presentations.

  8. An unusual presentation of galactosemia: Hemophagocytic lymphohistiocytosis

    Directory of Open Access Journals (Sweden)

    Ahmet Afşin Kundak

    2012-12-01

    Full Text Available Hemophagocytic lymphohistiocytosis (HLH is a rare life-threatening condition. Uncontrolled proliferation of activated lymphocytes secreting high amounts of inflammatory cytokines seems to be the main pathogenesis. The diagnosis of HLH can often be difficult. It may presents in many forms such as fever of unknown origin, hepatitis, acute liver failure, and sepsis-like illness. Here we present a newborn galactosemia case presented with HLH. Close monitoring of the diagnostic criteria of HLH during the course of galactosemia-associated hemophagocytosis, both before and after dietary treatment, should be performed in order to fully determine if the triggering mechanism is infection or accumulation of metabolites.

  9. Past and present trends of nuclear chemistry

    International Nuclear Information System (INIS)

    Matel, L.; Kuruc, J.

    2007-01-01

    This book represents not only the papers and lectures presented on the Seminar at the occasion of forty years of foundation of the Department of Nuclear Chemistry which took place on October 3 - 5, 2006 in Kezmarske Zlaby (High Tatras). It also contains the papers and presentations of post-graduate students and workers of the Department of Nuclear Chemistry as well as colleagues working in different field of nuclear chemistry and radioecology on various workplaces in the Slovak Republic, too. The book contains 17 papers, 15 presentations, photographs and 3 short video recording

  10. Gynaecomastia as the initial presentation of thyrotoxicosis

    Directory of Open Access Journals (Sweden)

    Triranjan Sanyal

    2012-01-01

    Full Text Available Gynaecomastia is a well-recognized manifestation of thyrotoxicosis occurring in a widely variable percentage. Some studies have reported the percentage to be high as 40% while others report it being around 10%. The variation can be attributed to the difference in the criteria used to define gynae-comaslia or racial factors. However, thyrotoxicosis presenting with gynaceomastia as an intial manifestation is very rare. Only handful case reports are found in literature. Here we present a case of 35-year-old Indian male, with thyrotoxcosis who presented initially with painful bilateral gynaceomastia.

  11. Offshore Wind Energy Market Overview (Presentation)

    Energy Technology Data Exchange (ETDEWEB)

    Baring-Gould, I.

    2013-07-01

    This presentation describes the current international market conditions regarding offshore wind, including the breakdown of installation costs, how to reduce costs, and the physical siting considerations considered when planning offshore wind construction. The presentation offers several examples of international existing and planned offshore wind farm sites and compares existing international offshore resources with U.S. resources. The presentation covers future offshore wind trends and cites some challenges that the United States must overcome before it will be able to fully develop offshore wind sites.

  12. Lymphogranuloma Venereum Presenting as a Rectovaginal Fistula

    Directory of Open Access Journals (Sweden)

    C. M. Lynch

    1999-01-01

    Full Text Available Lymphogranuloma venereum (LGV is a rare form of the sexually transmitted disease caused by Chlamydia trachomatis. In the United States, there are fewer than 350 cases per year. In a review of the world’s literature, there has not been a case reported in the last thirty years of a case ofLGV presenting as a rectovaginal fistula. We present a case of an otherwise healthy American woman who presented with a rectovaginal fistula. Although uncommon, LGV does occur in developed countries and may have devastating tissue destruction if not recognized and treated before the tertiary stage. Infect. Dis. Obstet. Gynecol. 7:199–201, 1999.

  13. Lymphogranuloma venereum presenting as a rectovaginal fistula.

    Science.gov (United States)

    Lynch, C M; Felder, T L; Schwandt, R A; Shashy, R G

    1999-01-01

    Lymphogranuloma venereum (LGV) is a rare form of the sexually transmitted disease caused by Chlamydia trachomatis. In the United States, there are fewer than 350 cases per year. In a review of the world's literature, there has not been a case reported in the last thirty years of a case of LGV presenting as a rectovaginal fistula. We present a case of an otherwise healthy American woman who presented with a rectovaginal fistula. Although uncommon, LGV does occur in developed countries and may have devastating tissue destruction if not recognized and treated before the tertiary stage. PMID:10449269

  14. Lichen planus pigmentosus: two atypical presentation

    Directory of Open Access Journals (Sweden)

    Falguni Nag

    2013-01-01

    Full Text Available Lichen planus pigmentosus (LPP is a chronic pigmentary disorder with variable pattern of presentation. We here by present two cases of LPP one with parallel band like pigmentation over abdomen sparing the abdominal skin creases and other with parallel band like pattern following the Blaschko’s lines over left side of the abdomen. Our cases are unique not only for its presentation but also for the pattern of distribution and LPP should be the differential diagnosis in any pigmentary disorders.

  15. Power point 2002 for successful presentation

    International Nuclear Information System (INIS)

    Moon, Insoo

    2002-01-01

    This book mentions power point 2002 for successful presentation, which deals with power point and presentation, all guide, the latest gear, for presentation, basic of power point 2002 such as slide, text compile, insertion of picture, figure and application of office guide, setting up new year plan using text like insertion text with various methods, compile effective text, and 200% application of tab, and addiction of pretty shape of characters, finishing of conversion of chinese character, and elimination of typographical error with spell checker.

  16. An unusual presentation of testicular tumor

    International Nuclear Information System (INIS)

    Amin, M.U.; Rahan, T.; Haq, A.U.; Aftab, P.

    2006-01-01

    A case of testicular choriocarcinoma is reported in which blood mixed stools and haemoptysis were the presenting manifestations as the patient never told about the testicular swelling to his parents. Orchidectomy was performed but the patient presented again with massive hematemesis due to gastric perforation secondary to gastric metastasis. The size of the testis at diagnosis was approximately 12 x 7cm. This was also unusual as testicular choriocarcinoma presents as a small mass. The patient eventually died of the complications within one month of diagnosis. (author)

  17. Moxibustion for Cephalic Version of Breech Presentation.

    Science.gov (United States)

    Schlaeger, Judith M; Stoffel, Cynthia L; Bussell, Jeanie L; Cai, Hui Yan; Takayama, Miho; Yajima, Hiroyoshi; Takakura, Nobuari

    2018-05-01

    Moxibustion, a form of traditional Chinese medicine (TCM), is the burning of the herb moxa (Folium Artemisiae argyi or mugwort) over acupuncture points. It is often used in China to facilitate cephalic version of breech presentation. This article reviews the history, philosophy, therapeutic use, possible mechanisms of action, and literature pertaining to its use for this indication. For moxibustion, moxa can be rolled into stick form, placed directly on the skin, or placed on an acupuncture needle and ignited to warm acupuncture points. Studies have demonstrated that moxibustion may promote cephalic version of breech presentation and may facilitate external cephalic version. However, there is currently a paucity of research on the effects of moxibustion on cephalic version of breech presentation, and thus there is a need for further studies. Areas needing more investigation include efficacy, safety, optimal technique, and best protocol for cephalic version of breech presentation. © 2018 by the American College of Nurse-Midwives.

  18. Late presentation for HIV care across Europe

    DEFF Research Database (Denmark)

    Mocroft, Amanda; Lundgren, Jens; Antinori, Andrea

    2015-01-01

    Late presentation (LP) for HIV care across Europe remains a significant issue. We provide a cross-European update from 34 countries on the prevalence and risk factors of LP for 2010-2013. People aged ≥ 16 presenting for HIV care (earliest of HIV-diagnosis, first clinic visit or cohort enrollment......) after 1 January 2010 with available CD4 count within six months of presentation were included. LP was defined as presentation with a CD4 count HIV diagnosis. Logistic regression investigated changes in LP over time. A total.......02-1.32), and a significant decline in LP in northern Europe (aOR/year later 0.89; 95% CI: 0.85-0.94). Further improvements in effective HIV testing strategies, with a focus on vulnerable groups, are required across the European continent....

  19. Demographics and presenting clinical features of childhood ...

    African Journals Online (AJOL)

    annaline

    predominance in adolescence and adulthood. The true incidence of ... Conclusion. There is a diverse array of presenting features in childhood SLE. There has .... difficult by the psychological problems associated with coping with a chronic ...

  20. Hydroelectric power in Romania. Past - present - future

    International Nuclear Information System (INIS)

    Bogan, V.

    1996-01-01

    This paper presents an analysis of the past and present situation in hydroelectric power (achievements, rates of commissioning and so on) and the future strategies for the hydroelectric power resource development in the conditions of a free market economy. At present the contribution of hydroelectric power in the Romania's total power balance is about 16,500 GW h/year which represents nearly 28 %. The theoretical hydroelectric power potential of Romania is 75,000 GW h/year while the technical potential, which could actually be developed, is only 40,000 GW h/year. Finally, there are presented the main directions in the hydroelectric power development up to the year 2020. (author) 3 tabs